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Published in Bobby Krishnachetty, Abdul Syed, Harriet Scott, Applied Anatomy for the FRCA, 2020
Bobby Krishnachetty, Abdul Syed, Harriet Scott
Surgical managementPalliative shunt: this is usually with a modified Blalock–Taussig shunt, directing blood from the subclavian artery to the pulmonary artery, allowing blood to flow to the lungs to receive oxygen, thus functioning like a patent ductus arteriosus.Corrective surgery: complete repair consists of two main steps – closure of the VSD with a patch and reconstruction of the right ventricular outflow tract on cardiopulmonary bypass. It is ideally performed in the first year of life, usually at 4–6 months of age. It is important to manage a hypercyanotic spell to reduce infundibular spasm, improve oxygenation and increase cardiac output.
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
Prostaglandin E2 infusion and balloon atrial septostomy are performed if severe cyanosis in the newborn period. A Blalock–Taussig shunt is then performed, followed later by a Fontan procedure (redirection of blood flow from the inferior vena cava to the right pulmonary artery with then functionally a univentricular heart).
Congenital heart disease
Published in Andrew R. Houghton, MAKING SENSE of Echocardiography, 2013
It’s very rare to see adults with untreated ToF, as fewer than 10 per cent of patients with untreated ToF survive to the age of 20 years. As a result, almost all the adults seen with a history of ToF will have undergone surgical correction. ToF is usually treated with primary repair (closing the VSD and relieving the RVOT obstruction) before the age of 1 year. However, where necessary it is possible to perform a modified Blalock–Taussig shunt procedure (placing a graft between the subclavian artery and the pulmonary artery) as a palliative measure – this does not fully correct the ToF, but it does increase blood flow to the pulmonary circulation.
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
The survival of patients with congenital heart disease has significantly improved over the past several decades. Hypoplastic left heart syndrome (HLHS) and other single ventricle heart lesions were uniformly fatal 40 years ago. Now, many patients with HLHS undergo staged palliation with survival ranging from 70% to 90% [1, 2]. The palliative approach includes stage 1 surgical palliation in the neonatal period with either the Norwood procedure with a modified Blalock-Taussig shunt (mBTS) or a right ventricle to pulmonary artery conduit (RV-PA conduit); or a hybrid procedure with stenting of the patent ductus arteriosus and bilateral branch pulmonary artery band placement. At approximately 4–6 months of age patients undergo stage 2 palliation, most commonly, with a superior cavopulmonary anastomosis (bidirectional Glenn procedure). This is followed by stage 3 palliation with the Fontan operation at generally 2–4 years of age. The stage 2 superior cavopulmonary anastomosis and stage 3 Fontan procedures have excellent surgical outcomes [3]. Mortality, however, is greatest following stage 1 palliation and within the first interstage period – the time between stage 1 and stage 2 operations [4].
Applications of computational fluid dynamics to congenital heart diseases: a practical review for cardiovascular professionals
Published in Expert Review of Cardiovascular Therapy, 2021
Gianluca Rigatelli, Claudio Chiastra, Giancarlo Pennati, Gabriele Dubini, Francesco Migliavacca, Marco Zuin
Migliavacca et al. [51] investigated the Norwood circulation by means of CFD analyzing the shunt pressure drop–flow relationships, varying shunt implantation angles, diameter, curvature, and input pulsatility and found, as expected, that shunt diameter was the main determinant of graft flow. The researchers found that most of the pressure drop occurred close to the proximal anastomosis, and curved grafts resulted in a lower pressure drop as compared with straight grafts, due to reduced flow-line skewness toward the lateral graft wall near the proximal anastomosis. Subsequently, the same research group compared the variants of the Norwood reconstructive surgeries with post-operative catheterization and Doppler data by carrying out a multi-scale CFD analysis [52]. The Norwood operation with a modified Blalock–Taussig shunt was compared with the right ventricle to pulmonary artery shunt modification. The model predicted that the right ventricle shunt would result in higher aortic diastolic pressure, decreased pulmonary arterial pressure, lower pulmonary to systemic flow, and higher coronary perfusion relative to the innominate artery-to-right pulmonary artery shunt. CFD by means of simulation of different diameter and length of shunt can be used to predict which configuration allows for the most favorable hemodynamic pattern [53] (Figure 5).
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.