Which of the following is a true surgical emergency in a newborn?
Total anomalous pulmonary venous connection (TAPVC) occurs in 1 to 2% of all cardiac malformations and is characterized by abnormal drainage of the pulmonary veins into the right heart, whether through connections into the right atrium or into its tributaries. Accordingly, the only mechanism by which oxygenated blood can return to the left heart is through an ASD, which is almost uniformly present with TAPVC. Unique to this lesion is the absence of a definitive form of palliation. Thus, TAPVC represents one of the only true surgical emergencies across the entire spectrum of congenital heart surgery.
The bidirectional Glenn procedure is used to correct:
Recognizing the inadequacies of the initial repairs for tricuspid atresia, Glenn described the first successful cavopulmonary anastomosis, an end-to-side right pulmonary artery (RPA)-to-superior vena cava (SVC) shunt in 1958, and later modified this to allow flow to both pulmonary arteries. This end-to-side RPA-to-SVC anastomosis was known as the bidirectional Glenn, and is the first stage to final Fontan repair in widespread use today. The Fontan repair was a major advancement in the treatment of congenital heart disease, as it essentially bypassed the right heart, and allowed separation of the pulmonary and systemic circulations.
Hypoplastic left heart syndrome is surgically treated with:
In 1983, Norwood and colleagues described a two-stage palliative surgical procedure for relief of hypoplastic left heart syndrome that was later modified to the currently used threestage method of palliation. Stage 1 palliation, also known as the modified Norwood procedure, bypasses the LV by creating a single outflow vessel, the neoaorta, which arises from the RV. More recently, Sano introduced a modification that includes arch reconstruction and placement of the shunt between the RV and pulmonary artery (Sano shunt), which diminishes the diastolic flow created by the classical B-T shunt and may augment coronary perfusion, resulting in improved postoperative cardiac function. A newer approach combines surgical and percutaneous techniques (hybrid procedure). The bilateral pulmonary arteries of surgically banded to restrict excess pulmonary blood flow after pulmonary vascular resistance drops and ductal stenting to maintain patency. The hybrid procedure does not require cardiopulmonary bypass.
The arterial switch operation for transposition of the great vessels is best performed:
The most important consideration is the timing of surgical repair, because arterial switch should be performed within 2 weeks after birth, before the LV loses its ability to pump against systemic afterload. In patients presenting later than 2 weeks, the LV can be retrained with preliminary pulmonary artery banding and aortopulmonary shunt followed by definitive repair. Alternatively, the unprepared LV can be supported following arterial switch with a mechanical assist device for a few days while it recovers ability to manage systemic pressures. Echocardiography can be used to assess left ventricular performance and guide operative planning in these circumstances.
Which of the following is NOT one of the components of the tetralogy of Fallot (TOF)?
The four features of tetralogy of F allot (TOF) are:
This combination of defects arises as a result of underdevelopment and anteroleftward malalignment of the infundibular septum.
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