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Ebstein’s anomaly of the tricuspid valve
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
One-and-half ventricular repair (bidirectional Glenn procedure) is usually performed in high-risk patients with a severely reduced size of the right ventricle, or a severely dysfunctional and enlarged right ventricle, extended atrialized right ventricle and long-standing atrial fibrillation.1,15 End-to-side anastomosis of the superior vena cava to the right pulmonary artery is performed in addition to the intracardiac repair (see Figure 15.2).16 It reduces venous return to the dysfunctional right ventricle by almost half, and optimizes preload to the left ventricle. Construction of the shunt in patients with left ventricular dysfunction is possible if left ventricular end-diastolic pressure is <15mmHg, the transpulmonary gradient is <10mmHg and the mean pulmonary artery pressure is <18–20mmHg.1
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].
Overview and surgical aspects of Uhls anomaly
Published in Expert Opinion on Orphan Drugs, 2020
Kotturathu Mammen Cherian, Lydia Jeris W, Mohamed Thayub
Fontan conversion can be done in patients with suitable hemodynamics [48]. Conversion from a bidirectional Glenn procedure to extra cardiac Fontan is rather a simple procedure compared to a single-stage lateral tunnel Fontan with fenestration [49,50]. Ultimately the procedure has to be decided depending on the collateral circulation.