One‐and‐a‐Half Ventricle Repair as a Surgical Alternative to Fontan Revision in an Adult

Padalino, Massimo A.; Maschietto, Nicola; Motta, Raffaella; Badano, Luigi P.; Stellin, Giovanni

doi:10.1111/jocs.12410

Cited by 5 publications

(2 citation statements)

References 15 publications

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“…Hypo-plastic right ventricle could be associated with different abnormalities such as pulmonary valve atresia and tricuspid valve atresia as well as other congenital defects such as interventricular septal defects. 6 The underdevelopment of the trabecular portion but normally developed pulmonary and tricuspid valves could lead to a reduction in the right ventricle size, characterized as isolated right ventricle hypoplasia, which is a rare disease with only a few cases reported. 7 The main cause of isolated right ventricle hypoplasia in most cases is unknown; in some cases, however, the cause appears to be familial.…”

Section: Discussionmentioning

confidence: 99%

Best management in isolated right ventricular hypoplasia with septal defects in adults

Khajali

Arabian

Aliramezany

2020

J Cardiovasc Thorac Res

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Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. Isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. It is associated with inter atrial septal defects leading to the right-to-left shunting of blood. Patients with isolated right ventricular hypoplasia usually have different and variable courses. In some patients, it is recognized in the perinatal period and necessitates prompt intervention; nonetheless, there are some reports of this anomaly in old age with no significant symptoms. In this report, we describe the clinical data and management of 6 adult cases with isolated right ventricular hypoplasia treated medically or surgically based on the severity of the disease and symptoms and then offer an in-depth discussion regarding this rare anomaly.

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Section: Discussionmentioning

confidence: 99%

Best management in isolated right ventricular hypoplasia with septal defects in adults

Khajali

Arabian

Aliramezany

2020

J Cardiovasc Thorac Res

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“…In this context, when complete venous rerouting could not be achieved, an ingenious combination of surgical procedures were adopted, such as hemi-mustard with bi-directional Glenn procedures, to offload the suboptimal right ventricle after a Rastelli correction. By this procedure, a partially pumping right ventricle is retained in the hemi-Fontan circuit created (one and a half ventricular repair) ( 8 , 9 ). Our previous published results had indicated that one-and-a-half ventricular repair was technically feasible for correction of ccTGA/LVOTO/cardiac malposition and had satisfied mid-term results ( 10 ).…”

Section: Introductionmentioning

confidence: 99%

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

Pang²,

Zhang³

et al. 2022

Front. Cardiovasc. Med.

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ObjectivesThis study was to assess the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli procedure) and Fontan pathway for correcting congenitally corrected transposition of great artery (ccTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malposition.MethodsIn this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malposition underwent the one-and-a-half ventricular repair (group A; 33 cases) and Fontan operation (group B; 41 cases) between October 2011 and March 2018. The Median follow-up time was 49 (20–84) and 42 (7–85) months in groups A and B, respectively. To estimate excise tolerance the 6-min walk test (MWT) was performed.ResultsNo in-hospital death. Compared with group A, group B have significantly less CPB, mechanical ventilation time, and intensive care unit stay, but prolonged pleural effusions developed more frequently in Group B. The survival probability was 90.2% (95% CI, 80.2–100%) and 97.2% (95% CI, 92–100%) at 7 years (p = 0.300) in group A and B. The probability of freedom from re-intervention were 80.6% (95% CI, 66.5–97.6%) and 97.2% (95% CI, 92–100%) at 7 years (p = 0.110). Longitudinal repeated measured echo data at every follow-up time shows that group A has more systemic ventricular EF% (p < 0.001) and less moderate systemic ventricular valve regurgitation (p < 0.001) compared with group B. Estimated by 6 MWT, group A has better outcomes for 6-min walk distance.ConclusionsFor correction of ccTGA with LVOTO and cardiac malposition, the one-and-a-half ventricular repair had superior midterm heart function and excise tolerance.

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One and half ventricle repair: rationale, indications, and results

Talwar

Siddharth

Choudhary

et al. 2018

Indian J Thorac Cardiovasc Surg

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One‐and‐a‐Half Ventricle Repair as a Surgical Alternative to Fontan Revision in an Adult

Abstract: We report a case of a young man with hypoplastic right ventricle, who presented with recurrent untreatable arrhythmias after a Bjork Fontan procedure in infancy. He underwent one-and-a-half ventricle repair as an alternative surgery to Fontan revision.

Cited by 5 publications

References 15 publications

Best management in isolated right ventricular hypoplasia with septal defects in adults

Best management in isolated right ventricular hypoplasia with septal defects in adults

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

One and half ventricle repair: rationale, indications, and results

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