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Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
A Norwood procedure is used as first-stage palliation for hypoplastic left heart syndrome. The aortic arch is enlarged with a patch, a side-by-side anastomosis is performed between the main pulmonary artery and the ascending aorta, an atrial septectomy is created, and a conduit is constructed to provide pulmonary blood flow. This may be either a modified Blalock–Taussig shunt (from the innominate artery to the pulmonary artery), or a Sano shunt (from the right ventricle to the pulmonary artery). Second-stage palliation is via the Glenn shunt, which is an end-to-side anastomosis between the superior vena cava and the right pulmonary artery, along with taking down of the previous shunt providing pulmonary blood flow. Third-stage palliation is via the Fontan procedure, which baffles the inferior vena cava to the right pulmonary artery. This may be accomplished with the lateral tunnel Fontan procedure, where an intra-atrial tunnel is created between the inferior vena cava and the cardiac end of the superior vena cava, along with reconnection of the superior vena cava to the undersurface of the pulmonary artery. Alternatively, an extracardiac Fontan procedure may be performed, whereby a polytetrafluoroethylene tube is sutured end to end to the inferior vena cava, with the other end of the tube sutured end to side to the pulmonary artery.
The Child With Suspected Congenital Heart Disease
Published in Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan, Diagnosing and Treating Common Problems in Paediatrics, 2017
Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan
In cases where an antenatal diagnosis has been made, to maintain patency of the ductus arteriosus, prostaglandin should be commenced immediately after birth. Where an antenatal diagnosis has not been made, neonates presenting with circulatory collapse should be resuscitated and commenced on prostaglandins. Surgical management of hypoplastic left heart syndrome involves three stages. The Norwood procedure is performed in the newborn period. The right ventricle is converted into the main ventricle for both the pulmonary and systemic circulation. A Blalock–Taussig shunt or Sano shunt is created to shunt blood to the pulmonary circulation. If coarctation is present, it is corrected. Post Norwood, children’s oxygen saturations are usually 70%–80%. Aspirin is given to prevent stent thrombosis. Children have a 75% survival rate at this time. At 3–5 months of age, a Glenn procedure is performed when circulation through the lungs no longer requires high ventricular pressure. The shunt is taken down and the superior vena cava is connected directly to the pulmonary circulation. The final stage takes place 18–36 months after the Glenn. The inferior vena cava is connected to the pulmonary circulation. At this stage, all deoxygenated blood flows passively into the lungs, so there is no mixing of oxygenated and deoxygenated blood.
Hypoplastic left heart syndrome (HLHS): molecular pathogenesis and emerging drug targets for cardiac repair and regeneration
Published in Expert Opinion on Therapeutic Targets, 2021
Anthony T Bejjani, Neil Wary, Mingxia Gu
The most common treatment for HLHS consists of three consecutive procedures done within the first 4 years of life: the Norwood, Glenn, and Fontan procedures. In brief, the Norwood procedure creates a new systemic circuit in the heart by redirecting blood from the left atrium into the right ventricle and connecting the aorta to the main pulmonary artery via a BT or Sano shunt [66,67]. This leads to the mixing of oxygenated and deoxygenated blood in the right ventricle. Despite this connection, pulmonary and systemic circulations are still in parallel. The Glenn procedure separates the superior vena cava from the RA and connects it directly to the pulmonary artery to be taken to the lungs, while the inferior vena cava remains connected to the RA. Finally, the Fontan procedure joins the superior and inferior venae cavae to the pulmonary trunk which is separated from the right ventricle, joining the left and right atria, and connecting the aorta to the right ventricle [68]. At the end of these procedures, the right heart would be solely responsible for the systemic circulation, with the venae cavae emptying directly into the pulmonary artery. A fenestration between the inferior vena cava and the RA remains to help reduce pressure to the lungs, while they adjust to the new circulation. At the end of the Fontan procedure, oxygen-rich and oxygen-poor continue to mix in the single-ventricle heart, while the pulmonary and systemic circulations are effectively in series.
Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome
Published in Fetal and Pediatric Pathology, 2019
Raya Safa, Richard Garcia, Ralph Delius, Gunjanpreet Kaur, Lara Youssef, Janet Poulik, Bahig M. Shehata
Hypoplastic left heart syndrome was a fatal disease until only a quarter century ago. The development of Norwood staged palliation greatly improved the outcome of the patients with hypoplastic left heart syndrome. However, despite the significant improvement in outcomes from surgical intervention, the Norwood procedure is a technical challenge and still carries significant mortality and morbidity [14]. The Norwood procedure is the first stage in a series of typically three heart surgeries to create a new functional systemic circuit in patients with single ventricle physiology. The Norwood procedure consists of creating an aortic arch, which is uniformly hypoplastic in hypoplastic left heart syndrome (HLHS), amalgamation of the proximal aorta and proximal pulmonary artery, and establishment of pulmonary blood flow by either a modified Blalock-Taussig shunt or a Sano shunt (right ventricle to pulmonary artery). An atrial septectomy is also performed to provide complete mixing at the atrial level and to allow unobstructed pulmonary venous return.
Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2022
Meredith C. G. Broberg, Ira M Cheifetz, Sarah T Plummer
Specific to the care of single ventricle patients following stage 1 palliation, two case series show advantageous results with beta-blocker use for rate control in patients with cyanosis due to proximal Sano shunt obstruction [50, 51]. In addition, a single center observational study of 51 patients, following the hybrid procedure for stage 1 single ventricle palliation, showed favorable weight gain with no significant side effects while receiving medical therapy primarily consisting of a beta-blocker (100% of patients), an ACEI (72% of patients), and mineralocorticoid inhibition (78% of patients) [52]. A literature review demonstrated no dedicated studies on the use of beta-blockers in the interstage single ventricle population.