Transplant-free survival after Norwood with B-T shunt (system to pulmonary) versus Sano shunt (RV to pulmonary shunt) in patients with hypoplastic left heart syndrome is:
The postoperative management of infants following stage 1 palliation is complex because favorable outcomes depend on establishing a delicate balance between pulmonary and systemic perfusion. Recent literature suggests that these infants require adequate postoperative cardiac output in order to supply both the pulmonary and the systemic circulations and that the use of oximetric catheters to monitor mixed venous oxygen saturation (Svo2) aids clinicians in both the selection of inotropic agents and in ventilatory management. Recent introduction of a modification that includes arch reconstruction and placement of the shunt between the RV and the pulmonary artery (Sano shunt) diminishes the diastolic flow created by the classical B-T shunt and may augment coronary perfusion, resulting in improved postoperative cardiac function. A recent prospective, randomized, multi-institutional trial sponsored by the National Institutes of Health, the Systemic Ventricle Reconstruction (SVR) trial, compared the outcomes of neonates having either a modified B-T shunt versus a Sano shunt. The SVR trial demonstrated that transplantation-free survival 12 months after randomization was higher with the Sano shunt than with the modified B-T shunt (74 vs 64%, P = 0.01). However, the Sano shunt group had more unintended interventions (P = 0.003) and complications (P = 0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (± standard deviation) follow-up period of 32 ± 11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P = 0.06) .
A premature infant is discovered at birth to have a patent ductus arteriosus with moderate respiratory distress. The infant does not improve after 48 hours of medical management with fluid restriction, diuretics, and respiratory support. The next step in management is:
Prostaglandins oppose contraction of the smooth muscle that obliterates the ductus. Indomethacin is a prostaglandin inhibitor and, given intravenously, leads to closure of the ductus in the premature infant. A national cooperative study found that indomethacin effected closure in 79% of 3559 patients studied. Although surgical closure of the ductus is surprisingly well-tolerated in these infants, operation should not be done unless this therapy does not close the ductus and symptoms are poorly controlled.
Which of the following is NOT a type of VSD?
VSD refers to a hole between the left and RV s. These defects are common, comprising 20 to 30% of all cases of congenital heart disease, and may occur as an isolated lesion or as part of a more complex malformation. VSDs vary in size from 3 to 4 mm to more than 3 em, and are classified into four types based on their location in the ventricular septum: perimembranous, atrioventricular canal, outlet or supracristal, and muscular.
During left thoracotomy for repair of patent ductus arteriosus the blood pressure is 70/22. Immediately after placement of a clip across the duct the blood pressure is:
Patent ductus arteriosus results in lower aortic diastolic pressure which increases the potential for myocardial ischemia and underperfusion of other systemic organs, while increased pulmonary blood flow leads to increased work of breathing and decreased gas exchange. Closure of the duct immediately increases diastolic systemic blood pressure, while leaving systolic pressure unchanged.
All of the following are true about truncus arteriosus EXCEPT:
The truncal valve is most commonly trileaflet (60%), but occasionally bicuspid and rarely quadricuspid (25%). Patients present in the neonatal period with signs and symptoms of congestive heart failure and mild to moderate cyanosis. A pansystolic murmur may be noted at the left sternal border and occasionally a diastolic murmur may be heard in the presence of truncal regurgitation. The presence of truncus arteriosus is an indication for surgery. Repair should be undertaken in the neonatal period or as soon as the diagnosis is established to prevent the development of pulmonary hypertension due to pulmonary over circulation. The presence of Eisenmenger physiology, which is found primarily in older children, is the only absolute contraindication to correction.
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