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Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Various degrees of ventricular hypoplasia may be seen in many of the congenital heart defects discussed in this chapter. True single-ventricle anatomy, in which a second hypoplastic ventricle is not able to be identified, is unusual but can be seen in some cases of double-inlet left ventricle. Single-ventricle physiology is more prevalent and refers to a patient who has undergone surgical Fontan palliation.
Cardiology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Initial surgery depends on the associated lesions. In the cyanosed infant, a palliative systemic to pulmonary anastomosis (e.g. Blalock shunt) may be required initially to improve pulmonary blood flow and hypoxia; banding is required when there is excessive pulmonary flow. Ultimately treatment for double inlet left ventricle, like all single ventricle circulations such as tricuspid atresia, pulmonary atresia with intact septum and hypoplastic left heart, aims for a total cavopulmonary circulation (Fontan) where the single ventricle pumps blood around the body and the pulmonary circulation drains by passive flow. Usually this is staged with a Glenn shunt preceding the total cavopulmonary connection. A Glenn shunt is typically superior vena cava to both pulmonary arteries (bidirectional) (Fig. 5.20). When the inferior vena cava is connected to the pulmonary arteries this is called a total cavopulmonary connection or Fontan operation.
Functionally single ventricle, Fontan procedure – univentricular heart/circulation
Published in Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček, Congenital Heart Disease in Adults, 2008
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček
This type of functionally single ventricle accounts for about 1.3% of all CHD. In double-inlet left ventricle (DILV), there are two atria; however, both atria empty into a single ventricle, which is a morphological left ventricle. The atrioven-tricular (AV) connection can be established by one single AV valve or by two (right and left) AV valves (Figures 15.4–15.6). The right- and left-sided AV valves are on the same plane, and these valves are neither morphological tricuspid nor mitral valves. The right AV valve orifice is overriding the ventricular septum and is predominantly (>50%) connected with the single left ventricle. The large left ventricle may communicate via the bulboventricular orifice with a hypoplastic infundibular right ventricle (Figure 15.7), which gives rise to the pulmonary artery (often hypoplastic or stenotic in adulthood). This arrangement protects the pulmonary vascular bed against excessive blood flow, allowing the patient to survive until adulthood without pulmonary hypertension. In patients with transposition of the great arteries, it is the aorta, which arises from the hypoplastic right ventricle.
Management for atrial arrhythmias in adults with complex congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2023
Victor Waldmann, Christophe Vô, Stefano Bartoletti, Francis Bessière, Marie Wilkin, Nabil Dib, Nicolas Combes, Paul Khairy
The ‘univentricular’ heart encompasses a spectrum of complex forms of CHD characterized by a predominantly single functional ventricle without the option of restoring biventricular physiology. The most prevalent type of univentricular heart is hypoplastic left heart syndrome. However, since staged surgery culminating in a Fontan circulation was introduced more recently for this defect, many adults with Fontan surgery currently have a single morphologic left ventricle instead (e.g. tricuspid atresia or double-inlet left ventricle). Older patients with Fontan surgery may have atrio-pulmonary connections associated with massive right atrial dilation. The proportion of adults with total cavopulmonary anastomoses, be they intra-atrial lateral tunnels or extracardiac conduits, is steadily rising.
Paradoxical nonreentrant tachycardia induced by iatrogenic atrioventricular block
Published in Acta Cardiologica, 2019
Mieke Roggen, Christophe Garweg, Rik Willems, Marc Gewillig, Joris Ector
A 5 week old male infant with complex congenital heart disease underwent cardiac catheterisation and balloon dilatation of a banded pulmonic artery. He was born with a univentricular heart: double inlet left ventricle with L-loop. He had undergone a surgical correction for aortic coarctation and pulmonic artery banding in the first week of his life.
Uni-ventricular pregnancy: a case report in a low resource country
Published in Journal of Obstetrics and Gynaecology, 2020
Bharat Bassaw, Dinesh Singh, Javed Chinnia, Abhinav Karan, Jonathan Ramsarran
In her history, she was diagnosed with a double-inlet left ventricle, hypoplastic right ventricle, transposition of the great arteries, coarctation of the aorta and moderate pulmonary stenosis. At ages 6 and 8, she underwent a Fontan Repair in the United States of America.