The dysfibrinogenemias consist of three types of fibrinogen disorders in which a critical blood clotting factor, fibrinogen, circulates at normal levels but is dysfunctional. Congenital dysfibrinogenemia is an inherited disorder in which one of the parental genes produces an abnormal fibrinogen. This fibrinogen interferes with normal blood clotting and/or lysis of blood clots. The condition therefore may cause pathological bleeding and/or thrombosis. Acquired dysfibrinogenemia is a non-hereditary disorder in which fibrinogen is dysfunctional due to the presence of liver disease, autoimmune disease, a plasma cell dyscrasias, or certain cancers. It is associated primarily with pathological bleeding. Hereditary fibrinogen Aα-Chain amyloidosis is a sub-category of congenital dysfibrinogenemia in which the dysfunctional fibrinogen does not cause bleeding or thrombosis but rather gradually accumulates in, and disrupts the function of, the kidney.Congenital dysfibrinogenemia is the commonest of these three disorders. Some 100 different genetic mutations occurring in more than 400 families have been found to cause it. All of these mutations as well as those causing hereditary fibrinogen Aα-Chain amyloidosis exhibit partial penetrance, i.e. only some family members with one of these mutant genes develop dysfibrinogenemia-related symptoms. While both of these congenital disorders as well as acquired dysfibrinogenemia are considered very rare, it is estimated that ~0.8% of individuals with venous thrombosis have either a congenital or acquired dysfibrinogenemia. Hence, the dysfibrinogenemia disorders may be highly under-diagnosed conditions due to isolated thrombotic events that are not appreciated as reflecting an underlying fibrinogen disorder.Congenital dysfibrinogenemia is distinguished from a similar inherited disorder, congenital hypodysfibrinogenemia.
As pulmonary heart disease progresses, most individuals will develop symptoms like:
Shortness of breath
Wheezing
Cyanosis
Ascites
Jaundice
Enlargement of the liver
Raised jugular venous pressure (JVP)
Third heart sound
Intercostal recession
Presence of abnormal heart sounds

Causes
The causes of pulmonary heart disease (cor pulmonale) are the following:

Acute respiratory distress syndrome (ARDS)
COPD
Primary pulmonary hypertension
Blood clots in lungs
Kyphoscoliosis
Interstitial lung disease
Cystic fibrosis
Sarcoidosis
Obstructive sleep apnea (untreated)
Sickle cell anemia
Bronchopulmonary dysplasia (in infants)

Pathophysiology
The pathophysiology of pulmonary heart disease (cor pulmonale) has always indicated that an increase in right ventricular afterload causes RV failure (pulmonary vasoconstriction, anatomic disruption/pulmonary vascular bed and increased blood viscosity are usually involved), however most of the time, the right ventricle adjusts to an overload in chronic pressure. According to Voelkel, et al., pressure overload is the initial step for changes in RV, other factors include:
Ischemia
Inflammation
Oxidative damage
Epigenetics
Abnormal cardiac energetics

Diagnosis
Investigations available to determine the cause of cor pulmonale include the following:
Chest x-ray – right ventricular hypertrophy, right atrial dilatation, prominent pulmonary artery
ECG – right ventricular hypertrophy, dysrhythmia, P pulmonale (characteristic peaked P wave)
Thrombophilia screen- to detect chronic venous thromboembolism (proteins C and S, antithrombin III, homocysteine levels)

Differential diagnosis
The diagnosis of pulmonary heart disease is not easy as both lung and heart disease can produce similar symptoms. Therefore, the differential diagnosis (DDx) should assess:
Atrial myxoma
Congestive heart failure
Constrictive pericarditis
Infiltrative cardiomyopathies
Right heart failure (right ventricular infarction)
Ventricular septal defect

Treatment
The treatment for cor pulmonale can include the following: antibiotics, expectorants, oxygen therapy, diuretics, digitalis, vasodilators, and anticoagulants. Some studies have indicated that Shenmai injection with conventional treatment is safe and effective for cor pulmonale (chronic).Treatment requires diuretics (to decrease strain on the heart).