This will help to examine the point of maximal impulse. Also, this will help to hear extra heart sounds (S3 or S4). With the patient sitting upright. With the patient seated, leaning forward and holding breath after exhalation. This will decrease the distance of the chest wall to the left ventricular outflow tract. By doing so this will help find the presence of an aortic regurgitation murmur. Radiation refers to where the sound of the murmur travels. The rule of thumb is that the sound radiates in the direction of the blood flow. Intensity refers to the loudness of the murmur with grades according to the Levine scale, from 1 to 6:Pitch may be low, medium or high. This depends on whether auscultation is best with the bell or diaphragm of a stethoscope. Quality refers to unusual characteristics of a murmur. For example, blowing, harsh, rumbling or musical. Mnemonics
A mnemonic to remember what characteristics to look for when listening to murmurs is SCRIPT: Site, Configuration (shape), Radiation, Intensity, Pitch and quality, and Timing in the cardiac cycle. The use of two simple mnemonics may help differentiate systolic and diastolic murmurs; PASS and PAID. Pulmonary and aortic stenoses are systolic while pulmonary and aortic insufficiency (regurgitation) are diastolic. Mitral and tricuspid defects are opposite. Interventions that change murmur sounds
Inhalation leads to an increase in intrathoracic negative pressure. This increases the capacity of pulmonary circulation, thereby prolonging ejection time. This will affect the closure of the pulmonary valve. This finding is also called Carvallos maneuver.
Adverse effects
Common adverse reactions may include nausea and elevated liver enzymes. Serious adverse reactions may include liver problems and low platelets. Transient elevated levels of cardiac troponin‑I were observed in clinical trials; the clinical importance of these findings is not known. However, cardiac toxicity was seen in studies of other animals.As the medication may reduce the platelet count, platelets may need to be checked before the medication is started, then weekly for the first month and every two weeks for the next two months until the level is back to baseline. Liver function should be monitored for three months after administration. Mechanism of action
SMA is a neuromuscular disorder caused by a mutation in the SMN1 gene, which leads to a decrease in SMN protein, a protein necessary for survival of motor neurons. Onasemnogene abeparvovec is a biologic drug consisting of AAV9 virus capsids that contains a SMN1 transgene along with synthetic promoters. Upon administration, the AAV9 viral vector delivers the SMN1 transgene to the affected motor neurons, where it leads to an increase in SMN protein. History
Onasemnogene abeparvovec was developed by the US biotechnology startup AveXis, which was acquired by Novartis in 2018, based on the work at the Institut de Myologie in France.The U.S. Food and Drug Administration (FDA) granted the application for onasemnogene abeparvovec-xioi fast track, breakthrough therapy, priority review, and orphan drug designations.