detected when pulmonary hypertension is present. This page intentionally left blank 598 CHAPTER 12 Congenital heart disease Surgical operations for CHD (1) Congenital heart surgery in adolescents and adults should o nly be under- taken in specialist centers. There are three categories: \200 patients who have not undergone prior operations \200 patients who have undergone previous palliative operations \200 patients who have undergone previous reparative procedures. Re-do sternot omy, preservation of myocardial performance, and atten- tion to pulmonary bed vascular abno rmalities and aorto-pulmonary col- laterals require careful prior planning and close co-ope ration between the surgeon, cardiac anesthetist, cardiologist, and intensive care team. Blalock \200\223Taussig shunt This is a procedure for systemic to pulmonary arterial shunt, using a sub- clavian arterial \202 ap (classical) or Gore-Tex tube (modi \201 ed) to increase pulmonary blood \202 ow and improve oxygenation in cyanotic congenital heart di sease. This is a palliative procedure which has superseded earlier operations such as Water ston shunt (ascending aorta to right pulmonary artery) or Pott \200\231s shunt (descending aorta to left pulmonary artery). Coarctation of the aorta This is relieved by either a subclavian \202 ap approach or dire ct end-to-end anastomosis. Occasionally, a bypass graft is required in adult patients, from the ascending to descending aorta. Glenn shunt The superior vena cava (SVC) is anastomosed to the ipsilateral pulmonary artery as a means of improving oxygenation in infants or young children. This is usually con \201 ned to patients with \200\230single \200\231 ventricles and univen- tricular circulat ions. In its classical form, the right pulmonary artery was detached from the main pulmonar y artery. The bidirectional Glenn shunt was fashioned such that blood from the SVC entered both pulmonary arteries. This page intentionally left blank 600 CHAPTER 12 Congenital heart disease Surgical operations for CHD (2) Tetralogy of Fallot (see Fig. 12.1) This is treated by clos ure of the ventricular septal defect with a patch of pericardium or Gore-Tex and relief of the RVOT obstruction by trans- annular patching, valvectomy, or placing a homograft/cond uit between the right ventricle and pulmonary artery. Rastelli operation (see Fig. 12.2) This is usually undertaken in patients with complex CHD, e.g. transposi- tion of the great arteries with large VSD. The VSD is closed by a large pa tch in such a way as to connect the left ventricle to the aorta, and a conduit or homograft connects the right ventricle to the pulmonary artery (PA). Ross operation This is an alternative to aortic valve replacement, which may be attrac- tiv e to younger patients, especially females. The diseased aortic valve is removed with the co ronary arteries detached. The patient \200\231s own pulmo- nary valve is resected and place d in the aortic position with the coronaries reattached. A homograft is placed between the right ventricle and the pulmonary artery. Fontan operation This represents the de \201 nitive palliation for patients with effective ly uni- ventricular circulations such as mitral or tricuspid atresia. Blood from the superi or and inferior vena cava is directed to the pulmonary arteries without the bene \201 t of a subpulmonary ventricle. Oxygenated blood returns to the systemic ventricle and is then p umped to the aorta. Mustard/Senning operations (see Fig. 12.3) These are life-saving innovations for treatment of patients with transposi- tion of the great arteries, which involve intra-atrial redirect ion of oxygen- ated and de-oxygenated blood to the systemic and pulmonary systems respectiv ely. The right ventricle serves as the systemic subaortic ventricle. The procedures have be en largely abandoned because of late problems with arrhythmias, pathway obstruction, and ve ntricular failure. Arterial switch This has largely superseded the Mustard/Senning procedure. The aorta and pu lmonary artery are relocated to their original positions, with re-anastomosis of the corona ry arteries to the neo-aorta. SURGICAL OPERATIONS FOR CHD (2) 601 RA Ao LA RA LV RV Fig. 12.1 Tetralogy of Fallot. Ao = aorta; LA = left atrium; LV = left ventricle; RA = righ t atrium; RV = right ventricle. Rastelli repair Ao Ao PA PA LA LA Pulmonary stenosis VSD RA RA LV RV LV RV Patch across RV prevents blood passing from RV to aorta Conduit connects RV to PA Fig. 12.2 The Rastelli operation. PA = pulmonary artery. 602 CHAPTER 12 Congenital heart disease 1 Aorta Art. pulm. V cava V. pulm. 2 Aorta Art. pulm. V. cava V. pulm. 3 Aorta Art. pulm. LA V. cava V. pulm. Fig. 12.3 The Senning operation. Redrawn from Koustantinov, IE et al. (2004). Atrial switch operation: past, present, and future. Annals of Thoracic Surgery , 77 : 2250 \200\2238, wi th permission. This page intentionally left blank 604 CHAPTER 12 Congenital heart disease Percutaneous transcatheter interventions for CHD Balloon atrial septostomy This is a life-s aving intervention in neonates with transposition of the great arteries in which an atrial communication is created by tearing a hole in the atrial septum using a balloon catheter. I t can be performed at the bedside under echocardiographic control. Pulmonary valvuloplasty This is the procedure of choice for patients of all ages with pulmo nary valvar stenosis. It is less successful if the valve is dysplastic (Noonan \200\231s sy n- drome). The pulmonary valve annulus is measured by echocardiography or angiography, and a balloon (100 \200\223120 % diameter) is used. It is one of the most effective transcathet er interventions. Aortic valvuloplasty This is of limited use in adults. If the aortic valve is pliable but n ot calci \201 ed, balloon dilatation may defer the need for de \201 nitive surgery but us ually at the expense of aortic regurgitation. The balloon size should not exceed the size o f the aorta at the site of the valve attachment. An antegrade approach via a trans-septal p uncture is advised, to reduce the incidence of valve damage. Atrial septal defect Most secundum atrial septal defects are amenable to percutaneous closu re. The Amplatzer septal occluding device is made of nitinol (a nicke titanium alloy wit h inherent shape memory) and comprises two disks with a self-centering waist. The defect ma y be balloon sized under transoesopha- geal echocardiographic (TOE) guidance. Defects up to 40 mm in size can be closed providing there are suf \201 cient rims ( > 5 mm) to the pulm onary veins and the attachment of the mitral valve. As a rule of thumb, patients with pulmo nary arterial pressures <70 mmHg can proceed to ASD closure. This is not suitable for any a trial defects other than those of the secundum variety, i.e. sinus venosus defects or parti al atrioventricular septal defects. Procedural complications are rare in expert hands. Anti platelet agents are administered for three to six months until the device has completely en dothelialized. Transient headaches are common in the weeks fol- lowing closure but serious complications are rare. These include transient ischaemic episodes/stroke, atrial arrhythmi as, and embolization of the device. Remember that concomitant electrophysiological procedur es may need to be undertaken in patients with signi \201 cant atrial arrhythmias. Ventricular septal defects Indications for closing defects in adults are limited, and the p rocedure is most frequently employed in patients with ischaemic VSD. Occasionally, \200 \230small \200\231 defects can produce volume overload of the left ventricle over time and require closure. Such defects may be amenable to transcatheter occlu- sion using the Amplat zer peri-membranous or muscular occluding devices. PERCUTANEOUS TRANSCATHETER INTERVENTIONS FOR CHD 605 This should only be undertaken in specialist centers. Complete heart block and device embol ization have been described. Coarctation of the aorta Surgical correction in the adult is a major challenge, with its as sociated risk of paraplegia. All patients have an aortopathy which renders the aortic tissu e very friable. Both native and re-coarctation of the aorta can now be successfully treated by transcatheter balloon dilatation and stenting. Covered stents may be required for nativ e coarctation, to reduce the incidence of dissection and rupture. MRI is invaluable in pati ent selec- tion and for selecting stent size. This intervention should only be under- taken in specialist centers where expert surgical help is readily available. Remember that ballo on dilatation of previous patch aortoplasty carries the highest risk of aortic rupture. Lif elong surveillance is necessary to detect aneurysm formation. Patent arterial duct This may occasionally present in later life. If small or medium-sized with no pulmonary hypertension, most can be successfully occluded with a variety of transca theter occluding devices or coils. Pulmonary valve implantation Percutaneous transcatheter implantation of a bovine jugular ve nous valve mounted within a platinum \200\223iridium stent into the right ventricular out \202 ow tract is now possible. This procedure, if it stands the test of time, will revoluti onize our approach to management of patients with pulmonary regurgitation following right h eart congenital surgery, offering as it does a non-surgical approach. These valves have bee n used in patients with right ventricle to pulmonary artery homografts which are leaking or sten- osed, as well as in native out \202 ow tracts. Increasingly, transcatheter aortic v alve implantation is employed in patients with signi \201 cant risk factors for surgery. 606 CHAPTER 12 Congenital heart disease Speci \201 c management issues Complications of cyanotic CHD Chronic hypoxaemia results in an increased red cell mass and total blood volume. This compensatory mechanism may initial ly enhance oxygen delivery; however, symptoms of hyperviscosity may develop. \200 Symptom s of hyperviscosity: headaches, visual disturbances (blurred or double vision), impaired alertness, fatigue, paraesthesia, tinnitus, myalgia, muscle weakne ss, restless legs, transient ischaemic events or stroke. Measure hemoglobin and red cell indices; most patients have a compensated erythropoesis with a stable hemoglobin. Therapeutic phlebotomy should be reserved for symptomatic patients e.g. hemoglobin > 20 g/d L or haematocrit > 65 % . Symptoms may be relieved by removal of 500 mL (maximum) of blood with concomitant \202 uid replacement with dextrose or saline. Phlebotomy should be perfo rmed no more than twice per annum as it increases the risk of stroke by causing iron de \201 ciency. Intravenous line \201 lters should always be used. Iron de \201 ciency in a polycythaemic patient should be aggressively treated with oral ir on supplementation (ferrous sulfate 600 mg/day), recheck hemoglobin 8 \200\22310 days later . \200 Oral iron is poorly tolerated by many patients (abdominal pain, diarrhea, constipa tion) and compliance is often poor. Furthermore, particularly in patients with systolic hea rt failure, oral iron is poorly absorbed and prolonged courses are needed to correct the de \201 ciency. This may also be the case in patients with cyanotic heart disease and high v enous pressure (liver congestion and high portal pressure). Therefore we prefer to use intr avenous iron therapy in many patients. How is iron sucrose given? \200 Iron sucrose: 5 mL vial containing 100 mg \200 The stan dard dose is 200mg (2 vials, 10 mL) \200 A small test dose, e.g. 2 mL should be given on the \201 rst occasion, followed by the remaining 8 mL after waiting 10 minutes It is quite safe to give it undiluted as a slow push over 10 minutes \200 Subsequent admi nistrations do not require a test dose Iron therapy, including the \201 rst dose, can be given safely in the outpatient clinic and this is the preferred setting. If patients live o utside London and are unable to attend the clinic three times a week, they should be admitt ed to the ward. Skin \200 Severe acne is common in cyanotic congenital heart disease. \200 It can act a s a source of infection and endocarditis. \200 Treat aggressively. Gallstones \200 Comm on in cyanosed patients. \200 Bilirubin is a product of hemoglobin breakdown. \200 Susp ect if the patient presents with severe abdominal pain/discomfort. SPECIFIC MANAGEMENT ISSUES 607 Renal function \200 Reduced glomerular \201 ltration rate. \200 Increased levels of c reatinine. \200 Abnormalities in clearance of uric acid frequently accompany chronic cyanosis, and gout, sometimes frank, may result. Cautious use of contrast in angiography is advised, and always pre-hydrate. Non-cardiac surgery \200 This should be undertaken in specialist centers with care delive red by a multidisciplinary team. Use local anesthesia if possible; otherwise use a cardiac anesthetist with experience in CHD. Consider preoperative phlebotomy if hemoglobin > 20 g/dL or haematocrit > 65 % . Air line \201 lters should be employed. Antibiotic prophylaxis New guidelines have been released by the National Institute for Heal th and Clinical Excellence (NICE). Antibiotic prophylaxis is no longer recom- mended for de ntal procedures. However, there are differences between these recommendations and those fro m the European Society of cardi- ology (ESC) and America. Many cardiologists still recommen d antibiotic prophylaxis for patients with a previous history of endocarditis or with a mec hanical valve. Patient attention to maintaining good dental hygiene is essential. Arrhythmia \200 Arrhythmia is an important cause of morbidity and mortality and major cau se for hospitalization of CHD patients. It may lead to signi \201 cant haemodynamic deteri oration and is poorly tolerated. It may be due to: underlying cardiac defect, e.g. atrial isomerism \200 part of natural history, e.g. haemo dynamic changes such as chamber enlargement or scarring residual postoperative abnormalities. Management Specialist advice is essential. New presentation demands a complete haemodynamic assessment including electrophysiological investigation. Correction of any underlying post operative haemodynamic abnormality may be one of the most important therapeutic interventio ns. \200 Record a 12-lead ECG. \200 Administer IV adenosine whilst recording the ECG. T he mode of termination of the tachyarrhythmia may give very useful information on its etiology, e.g. p re-excitation. Urgent DC cardioversion may be required if haemodynamic compromise is present. Ideally, TOE should rule out intracardiac thrombus. If the patient i s on warfarin, always check the international normalized ratio (INR) as well as the serum e lectrolytes prior to cardioversion. 608 CHAPTER 12 Congenital heart disease Pregnancy The majority of women with CHD can tolerate pregnancy. But \200 Congenital heart disease is now the major cause of maternal cardiac death in dev eloped countries, All need specialist counseling before embarking on pregnancy. Cardiologists are woefully ill-prepared to give such advice \200\224 pediatric cardiologists sometimes more so! All young women of child-bearing age should undergo a detailed clinical and haemodynamic assessment, which should include a formal exercise test and detai led cross-sectional echocardiography with emphasis on systemic ventricular function. They s hould be reviewed by a cardiologist with a special interest in maternal cardiology. \200 All women with complex CHD should be closely monitored in specialist centers, where delivery should take place. Multidisciplinary input is essential. Pregnancy is contraindicated in some CHD patients. Women with CHD in whom pregnancy is contraindicated \200 Pulmonary hypertension e.g. Eisenmenger syndrome (50 % maternal and fetal mortality; maternal death may occur up t o two weeks post-partum) Heart failure, e.g. secondary to failing Fontan circulation \200 Women with severe cyanos is and polycythaemia: if saturation is less than 80 % at rest and hemoglobin is greater than 18 g/dL, there is a greater incidence of miscarriage and intrauterine growth retardation severe outfow tract obstruction, e.g. severe aortic stenosis Contraception \200 Should be tailored to the patient and her cardiac condition \200 For patients at risk of venous thromboembolism, newer progesterone- only pils (Cerazette ) or implants (Nexplanon ) are advised. Emergency contraception such as Levonelle can be used when no contraception has been use d, or when the usual method has failed. It can be taken by all patients. This page intentionally left blank 610 CHAPTER 12 Congenital heart disease Speci \201 c conditions (1) Atrial septal defect This is one of the commonest lesions, pre senting for the \201 rst time in adult life. It is often missed as clinical signs of \201 xed splitting of the second heart sound can be dif \201 cult to appreciate. It may pr esent for the \201 rst time with new-onset atrial \202 utter or \201 brillation. Cons ider the need for electrophysi- ological interventions, e.g. Maze procedure for atrial \201 brillation. Shunting tends to increase with aging as the left ventricle stiffens. Clos ure is indi- cated in all symptomatic patients few are truly asymptomatic when assess ed by cardiopulmonary testing! There is no upper age limit. The vast majority of secundum d efects are amenable to transcatheter closure. Pulmonary hypertension needs to be excluded b ut most patients with PA pressures <70 mmHg can safely undergo closure. Atrial arrhythmias are one of the common late medical problems. All other types such as sinus venosus defect ( defect in the upper atrial septum associated with over- riding of the SVC and anomalous rig ht pulmonary venous drainage) and partial AV septal defect require surgical closure. A sinu s venosus defect represents a major surgical challenge involving redirection of the anoma- lous pulmonary vein(s). The risk of endocarditis is very low. Antibiotic prophylaxis is not indicated. Ventricular septal defect The majority are small as they are usually repaired in childhood. Eisenmenger syndrome (pulmonary vascular disease associated with reverse, right-to-left sh unting via intracardiac communication \200\224 VSD, ASD, patent ductus arteriosus (PDA)) i s becoming increasingly rare. VSD should be closed if there is signi \201 cant shunt, prev ious endocarditis, development of aortic regurgitation, or RVOT obstruction. It is always i mportant to demonstrate reversibility of pulmonary hypertension prior to interven- tion. It may be amenable to surgical or transcatheter closure. Ventricular arrhythmias may occur la te during follow-up. Atrioventricular septal defect This is a common defect in patients with Down \200\231s synd rome. If unoper- ated, pulmonary vascular disease (Eisenmenger syndrome) develops. Left AV valve regurgitation/stenosis are the major determinants of need for further interventions. Valve replacement may be necessary. Pulmonary stenosis This may present for \201 rst time in adult life. It can be successful ly treated by balloon valvuloplasty, which should be undertaken if the valve gradient excee ds 30 mmHg on Doppler echocardiography. Surgery is reserved for calci \201 ed and dysplast ic valves. The outlook is excellent. The risk of endocarditis is low. SPECIFIC CONDITIONS (1) 611 Left ventricular out \202 ow tract obstruction Aortic valve stenosis is especially common in association with a bicuspid valve. This is a complex lesion and an aortopathy is commonl y present. There is a risk of aortic dilatation and dissection. The degree of stenosis or r egurgitation may increase over time. Effort intolerance, chest pain, or syncope, especially with exertion, need to be taken seriously and interven- tion considered. It may occasional ly be amenable to balloon valvuloplasty but more commonly requires surgical intervention. A ortic valve replace- ment (lifelong anticoagulation) or homograft insertion/Ross operation (no anticoagulation but potential need for further interventions, critically operator depen dent) can be offered. Subaortic stenosis is uncommon (no ejection click) and requires surgi cal resection if severe or the patient is symptomatic. Recurrence is possible. Supravalvar stenosis is commonly seen in patients with Williams \200\231 syndrome. It may be associated with mul- tiple stenoses in the origins of the coronary arteries and the aortic arch arter ial branches. Reconstructive surgery is complex. Coarctation of the aorta Most patients seen in adult clinics will have already undergone su rgery. Some may present for the \201 rst time with hypertension. Always feel the femoral pulses! Consider Turner \200\231s syndrome in female patients. Coarctation of the aorta is a complex lesion usually associated with a bicuspid aortic valve (ejection click at the ape x) and aortopathy. During follow-up, upper (right arm) and lower limb BP should be recorded and evidence of left ventricular hypertrophy (LVH) on ECG and cross-sectional echocardiogr aphy should be noted. Re-coarctation may occur over time and should be suspected if there a re reduced femoral pulses or radiofemoral delay on examination. Doppler echocardiography ca n help by revealing an increased systolic velocity signal with forward \202 ow through th e site of re-coarctation during diastole. MRI with three-dimensional reconstruction is the imaging modality of choice. Transcatheter balloon dilatation and stenting is feasible in ma ny patients, thus avoiding the risks of thoracic aortic surgery. Lifelong surveillance is n ec- essary to rule out aneurysm formation or recurrence. Anomalous pulmonary venous drainage Many patients will undergo surgery in early childhood. This may occur in association with Scimitar syndrome. If the patient is symptomatic (short- ness of breath, arrhythmia) and there is evidence of right heart volume overload, for exam ple on the cross-sectional echocardiogram, consider surgery. There is a low risk of endocar ditis. 612 CHAPTER 12 Congenital heart disease Speci \201 c conditions (2) Transposition of the great arteries The aorta arises from the right ventricle, and the pulmonary artery from the left ventricle. This is the commonest cy anotic cardiac lesion presenting in the \201 rst few days of life. In the absence of mixi ng at cardiac or great arterial level, this condition is uniformly lethal. The majority of adult survi- vors have undergone intra-atrial repair by Mustard or Senning procedure. These produced excellent early survival but signi \201 cant late morbidity and mortality. Commo n late problems are atrial arrhythmias ( \202 utter/ \201 brilla- tion), venous baf \202 e narrowing, and right ventricular failure with tricuspid regurgitation. Arrhythmias are p oorly tolerated and prompt restoration to sinus rhythm is essential. Baf \202 e stenosis c an be relieved by transcatheter balloon dilatation/stenting. Ventricular failure requires i ntensive medical therapy but ultimately may necessitate cardiac transplantation. Patients w ho have undergone the arterial switch procedure are now reaching early adulthood. Problems with coronary arterial stenoses at the site of re-anastomosis to the neo-aorta have been id enti \201 ed in some. Tetralogy of Fallot This comprises a large non-restrictive VSD, over-riding aorta, right RV OT obstruction, and right ventricular hypertrophy. This is the com- monest cyanotic cardiac lesion presenting outside the neonatal period. If it presents with critical cyanosis in th e neonatal period, the patient may undergo a palliative Blalock \200\223Taussig shunt prior to complete repair in early childhood. Many centers now opt for de \201 nitive repair eve n in the \201 rst few months of life. The vast majority of adults will have undergone som e form of repair with surgical closure of the septal defect and relief of the RVOT obstruct ion. Arrhythmias are an inevitable consequence of surgery (scar- ring, ventriculotomy, and pulmonary regurgitation). Late arrhythmias (both atrial and ventricular) and sudden death o ccur. Pulmonary regurgitation, ignored for many years as a benign condition, is now known t o be one of the most important causes of morbidity and mortality in patients following RVOT surgery. The QRS duration > 180 ms on the ECG has been used as a marker for patients at ri sk of arrhythmia and late sudden death. Patients should be assessed by exercise testing and MRI. A percutaneous approach to pulmonary valve implantation is available, using a bovine jugular venous valve mounted within a platinum \200\223iridium stent, and is suitable for p atients with severe pulmonary regurgitation and out \202 ow tracts <20 mm in size. Branch pulmonary arterial stenoses should be treated aggressively by catheter intervention. Surgic al insertion of a homograft valve is currently performed, with perioperative mortality less than 1 % in specialist centers. The majority of such conduits need replacing within a 10-y ear period, i.e. there is a need for multiple re-operations. Aortic regurgitation may also occur. SPECIFIC CONDITIONS (2) 613 Fontan patients This is the de \201 nitive palliation for patients born with \200\230sing le ventricle \200\231 physiology, in which all the systemic venous return is directed back to the lungs without the bene \201 t of a subpulmonary ventricle, e.g. mitral/ tri- cuspid /pulmonary/aortic atresia. It produces excellent early and mid-term survival but late failu re occurs even in the most carefully selected candi- dates. The late problems comprise atri al arrhythmias including sinus node dysfunction and heart block, AV valve regurgitation, ve ntricular failure, venous obstruction, and protein-losing enteropathy (<50 % 5-year sur- vi val). The failing Fontan is a major challenge, and transplantation or con- version to a mor e streamlined modi \201 cation with concomitant arrhythmia surgery should be considered. Cardiac transplantation is rarely undertaken in adults with CHD. This relates to lack of or gans, prior antibodies, complex anatomy, previous sternotomies, and collaterals with risk o f bleeding. There is a lack of suitably quali \201 ed surgical teams. Heart \200\223lung t ransplantation is rarely if ever undertaken for patients with either complex cyanotic CHD o r Eisenmenger syndrome. Congenitally corrected transposition of the great arteries Cyanosis is present if there is associated VSD and pulmonary stenosis or if there is pulmonary vascular disease with VSD an d no pulmonary stenosis. This is a rare lesion with prolonged survival in some, if there is an isolated lesion. There is an ongoing risk of complete heart block (2 % per annum). AV v alve regurgitation in association with systemic right ventricular failure is a major long-t erm problem. Ebstein \200\231s anomaly of the tricuspid valve This condition is associated with apical d isplacement of the tricuspid valve and is associated with atrial communication. Cyanosis oc curs via right-to-left shunt at atrial level. There is a wide spectrum of presenta- tion, d epending on the severity of tricuspid valve regurgitation and associ- ated anomalies. In in fancy, this presents with heart failure and cyanosis, and prognosis is poor. In the older c hild or young adult, the murmur may be an incidental \201 nding, and mild forms can be as ymptomatic. Atrial arrhythmias are common and associated with pre-excitation (accessory pat hways). Intervention is warranted if symptomatic with heart failure, severe tricuspid regur gitation, or arrhythmias. Newer surgical techniques involving reconstruction of the tricusp id valve have been introduced, with superior outcomes. Tricuspid valve replacement/repair c an be offered as a last resort. Interatrial communications causing cyanosis/paradoxical emb oli can be closed percutaneously if haemodynamic assessment permits. 614 CHAPTER 12 Congenital heart disease Extra-cardiac complications Polycythaemia: Chronic hypoxia stimulates erythropoietin produc tion and erythrocytosis. The \200\230ideal \200\231 hemoglobin level is 717 \200\22318 g/d L; some centers advocate venesection to control the haematocrit and prevent hyperviscosity syndrome. Follow local guidelines. Generally consider phlebotomy only if moderate or severe symptoms of hyperviscosity are present and haematocrit > 65 % . Remove 500 mL of blood ove r 30 \200\22345 minutes and replace volume simultaneously with 500 \200\2231000 mL saline, or salt-free dextran (if heart failure). Avoid abrupt changes in circulating volume. If hyp erviscosity symptoms are the result of acute dehydration or iron de \201 ciency, venesecti on is not required and the patient must be rehydrated and/or treated with iron. Renal disease and gout: Hypoxia affects glomerular and tubular function, resulting in prote inuria, reduced urate excretion, increased urate reabsorption, and reduced creatinine clear ance. Overt renal failure is uncommon. Try to avoid dehydration, diuretics, and radiographi c contrast. Asymptomatic hyperuricaemia does not need treatment. Colchicine and steroids ar e \201 rst-line agents for treatment of acute gout. Non-steroidal anti-in \202 ammatory drugs (NSAIDs) should be avoided. Sepsis: Patients are more prone to infection. Skin acne is common, with poor healing of sca rs. Skin stitches for operative procedures should be left in for 7 \200\22310 days longer t han normal. Dental hygiene is very important due to the risk of endocarditis. Any site of s epsis may result in cerebral abscesses from metastatic infection or septic emboli. Thrombosis and bleeding are multifactorial and are caused by a combination of abnormal plat elet function, coagulation abnormalities, and polycythaemia. Prothrombin time (PT) and acti vated partial thromboplastin times (aPTT) values may be elevated and secondary to a fall in factors V, VII, VIII, and X. Both arterial venous thromboses and haemorrhagic complicat ions (e.g. petechiae, epistaxes, haemoptyses) can occur. Dehydration or oral contraceptives are risk factors for thrombotic events. Spontaneous bleeding is generally self-limiting. I n the context of severe bleeding, general measures are effective, including platalet transf usion, fresh frozen plasma (FFP), cryoprecipitate, and vitamin K. Aspirin and other NSAIDs should generally be avoided to decrease the chances of spontaneous bruising/bleeding. Primary pulmonary problems include infection, infarction, and haemor- rhage from ruptured a rterioles or capillaries. Stroke: Can be both thrombotic and haemorrhagic. Arterial thrombosis, embolic events (parad oxical emboli in RlL shunt) and injudicious phlebotomy lead to spontaneous thrombosis. Haem ostasis problems, especially when combined with NSAIDs or formal anticoagulation, can lead to haemorrhagic stroke. Any injured brain tissue is also a nidus for intracranial infection /abscess formation. Complications secondary to drugs, investigations, surgery: Avoid abrupt changes in BP or sy stemic resistance. Contrast agents may provoke EXTRA-CARDIAC COMPLICATIONS 615 systemic vasodilatation and cause acute decompensation. They may also precipitate renal fai lure. Before non-cardiac surgery, try to optimize haematocrit and haemostasis by controlled phlebotomy and replacement with dextran. High- \202 ow oxygen is important before and aft er surgery. Extreme precaution is required with IV lines. Arthralgia: Is mainly due to hypertrophic osteoarthropathy. In patients with R l L shunt, m egakaryocytes bypass the pulmonary circulation and become trapped in systemic vascular beds , promoting new bone formation. Haemoptysis is common. Most episodes are self-limiting and precipitated by infection. Diffe rentiation from pulmonary embolism may be dif \201 cult. Try to keep the patient calm and ensure adequate BP control. Give high- \202 ow oxygen by mask. If there is clinical suspic ion of infection (fever, sputum production, leukocytosis, raised C-reactive protein (CRP), etc.) start broad-spectrum antibiotics. Ventilation \200\223perfusion (VQ) scan may help in the diagnosis of pulmonary embolism but is often equivocal. Avoid aspirin and NSAIDs as th ese exacerbate the intrinsic platelet abnormalities. There is anecdotal evidence for the us e of low-dose IV heparin, dextran 40 (500 mL IV infusion q4 \200\2236h), Arvin (reduces plasma \201 brinogen by cleaving \201 brin), or low-dose warfarin therapy for reducing thrombotic tendency in these patients. Severe pulmonary hemorrhage may respond to aprotinin or tranexamic acid. Breathlessness may be due to pulmonary oedema or hypoxia (increased shunt) secondary to che st infection or pulmonary infarction. Do not give large doses of diuretics or nitrates as t his will drop systemic pressures and may precipitate acute collapse. Compare CXR to previou s \201 lms to try to assess if there is radiological evidence of pulmonary oedema. The JV P in patients with cyanotic CHD is typically high and should not be used as a sole marker o f heart failure. Overall, patients need a higher \201 lling pressure to maintain pulmonar y blood \202 ow. Give high- \202 ow oxygen by mask. Start antibiotics if there is a clin ical suspicion of infection. Give oral diuretics if there is evidence of pulmonary oedema o r severe right heart failure. Monitor haematocrit and renal function closely for signs of o ver-diuresis. Effort syncope should prompt a search for arrhythmias, in particular ventricular tachycardi a (VT) (Holter monitor), severe valve disease, or signs of overt heart failure. Treat as ap propriate. Chest pain may be secondary to pulmonary embolism or infarction (spontaneous thrombosis), p neumonia, ischaemic heart disease, or musculoskeletal causes. It requires careful evaluatio n with the conventional diagnostic modalities already described. This page intentionally left blank 617 Chapter 13 Multisystem disorders Coronary artery disease in patients with rheumatologic diseases 618 Rheumatoid arthritis 620 Systemic lupus erythematosus 622 Antiphospholipid syndrome 624 Neo natal lupus 625 Systemic sclerosis 626 Pulmonary hypertension and connective tissue disease s 626 Polymyositis and dermatomyositis 627 Mixed connective tissue disease 627 Sj gren \200\231s syndrome 627 Takayasu arteritis 628 Giant cell (temporal) arteritis 629 Kawasaki disease 630 Small- and medium-vessel vasculitis 632 Small vessel vasculitis 632 Ankylosing spondylitis 634 Marfan syndrome 636 Ehlers \200\223Danlos syndrome 640 Rheumatic drug thera py and the heart 642 Erectile dysfunction 644 Chronic kidney disease as a cardiovascular ri sk factor 646 CKD: which examinations are appropriate? 648 Side-effects of cardiological in vestigations in patients with CKD 649 Prevention and treatment of cardiovascular sequelae in renal patients 650 618 CHAPTER 13 Multisystem disorders Coronary artery disease in patients with rheumatologic diseases Origins \200 Atherosclero sis, thrombosis, arteritis, vasospasm Epidemiology Systemic lupus erythematosus (SLE) \200 The prevalence of ischaemic heart di sease (IHD) is 6 \200\22310 % (true prevalence of subclinical disease not known). Women with SLE are overall 5 \200\2236 times more likely to have a myocardial infarction (MI) than non-lupus women of similar age. Rheumatoid arthritis (RA) \200 Incidence 4.8 \200\2235.9/1000 person-years. Patients with RA are 2 \200\2233 times more likely than matched controls to have a MI, after controlling for traditional risk factors. Epicardial disease is more common than microvascular disease. \200 Patients with acute co ronary syndrome (ACS) have a twofold recurrence rates risk and mortality compared with age- and sex-matched controls. Coronary arteritis is uncommon; it affects epicardial and small/ medium-sized intramyocardial arteries and usually presents as ACS. In \202 ammation and atherogenesis \200 There is evidence that in \202 ammation has a f undamental role in the initiation and progression of atherosclerosis. Increased risk of IHD cannot be fully explained by traditional atherogenic risk factors (hypertension, hyperlipidaemia, diabetes mellitus, smoking, obesit y, sedentary lifestyle). Disease or treatment-related risk factors: elevated homocysteine, glucocorticoid use, renal disease (SLE), seropositive disease (RA), and non-steroidal anti-in \202 ammatory drug (N SAID) use (probably by increasing blood pressure). Differential diagnosis of chest pains \200 IHD: maintain lower than normal threshold for investigating and appreciate it as a cause in younger patients Primary coronary artery thrombosis (antiphospholipid syndrome) \200 Upper gastrointestina l problems associated with NSAID use \200 Costochondritis \200 Osteoporotic rib/vertebr al fracture \200 Pleuropericarditis \200 Large-vessel vasculitis causing aortic dissect ion or aneurysm Management \200 Physician awareness and patient education \200 Manage traditional risk factors: aggressive blood pressure control, lipid- lowering agents, smoking cessation CAD IN PATIENTS WITH RHEUMATOLOGIC DISEASES 619 Individualized aerobic exercise program \200 Disease-speci \201 c potential risk factors : reduce homocysteine levels (consider folate supplementation), minimize glucocorticoid use , recognize thrombotic potential; consider antiphospholipid antibodies See Table 13.1. Table 13.1 Cardiac manifestations of rheumatic diseases e s a e s i d m e t s y s n o i t c u d n o C t o o r l a i d r a c o y M l a i d r a c i r e P y r a n o r o C r a l u v l a V e s a e s i d e s a e s i d e s a e s i d e s a e s i d e s a e s i d c i t r o A Ankylosing spondylitis + + + + + + + Beh et \200\231s disease + + + + Churg \200\223Strauss syndrome + + Giant cell arteritis + + + + + Kawasaki \200\231s disease + + + Neonatal lupus erythematosus + + + Polyarteritis nodosa + Polymyositis/dermatomyositis + + + Reiter \200\231s syndrome + Rheumatoid arthritis + + + + + + + + Adult Still \200\231s disease + + + + + Systemic lupus erythematosus + + + + + + + + + Systemic sclerosis + + + + + + Takayasu \200\231s arteritis + + + + + + + + Wegener \200\231s granulomatosis + Taken from Manzi S, Kao AH, Wasko MM Oxford Textbook of Rheumatology , Chapter 1.3.4 The ca rdiovascular system. Oxford: Oxford University Press. 620 CHAPTER 13 Multisystem disorders Rheumatoid arthritis \200 RA is a multisystem in \202 ammatory autoimmune disease charac terized by a symmetrical and progressive deforming, peripheral arthropathy. Prevalence 1 % ; male:female = 1:3, peak onset in the \201 fth decade. Clinical features \200 Constitutional symptoms such as fever, malaise and fatigue \200 Symmetrical polyarthritis with insidious or abrupt onset \200 Early morning and rest stif fness \200 Subcutaneous nodules \200 Pleuritis, interstitial lung disease, intrapulmona ry nodules \200 Scleritis, episcleritis, dry eyes Cardiac disease Coronary artery disease \200 See b p. 618. Pericarditis \200 50 % of ca ses have a benign \201 brinous pericarditis with effusions seen in 40 % of cases on echocardiography