Successful Repair of the Right Atrial Isomerism, Double Outlet Right Ventricle, Cornmon Atrioventricular Canal, Pulmonary Stenosis, and Total Anomalous Pulmonary Venous Connection

Introduction: The purpose of this study was to report our experience in the surgical reconstruction of the right ventricular outflow tract in double outlet right ventricle with a major coronary artery crossing the right ventricular outflow tract in the presence of mirror image-dextrocardia.Methods: From January 2005 to December 2019, 19 double outlet right ventricle patients (median age 4 years) with mirror image-dextrocardia and a major coronary artery crossing the right ventricular outflow tract received surgical repair. An autologous pericardial patch was used to enlarge the right ventricular outflow tract in four patients without pulmonary stenosis and three patients with mild pulmonary stenosis. A valved bovine jugular venous conduit was added to a hypoplastic native pathway in nine patients, among which six patients with moderate pulmonary stenosis received small-sized bovine jugular venous conduit implantation (diameter ≤ 16 mm). In comparison, a large-sized bovine jugular venous conduit (diameter >16 mm) was adopted in a total of three patients with severe pulmonary stenosis. Finally, three patients with preoperative pulmonary hypertension (mean pulmonary artery pressure ≥40 mmHg) did not undergo further intervention of right ventricular outflow tract due to the adequate outflow tract blood flow.Results: There was no hospital mortality. One patient with sub-pulmonary ventricular septal defect and concomitant severe pulmonary hypertension died from respiratory failure 11 months after the operation. Kaplan-Meier survival was 94% at 5, 10 years. Within a mean echocardiographic follow-up of 6.9 ± 3.6 years, a total of two patients received reintervention due to valvular stenosis of the bovine jugular venous conduit (pressure gradient > 50 mmHg at 4 and 9 years) after surgical operation. Actuarial freedom from reoperation was 90 and 72% at 5 and 10 years, respectively. During the last echocardiographic follow-up phase, all the survivors were in NYHA class I.Conclusions: Double outlet right ventricle with mirror image-dextrocardia is a rare and complicated congenital cardiac malformation. Surgical reconstruction of the right ventricular outflow tract should be individualized based on the degree of pulmonary stenosis and the specific anatomical features of each patient. Reconstructing the pulmonary artery using the various sizes of valved bovine jugular venous conduit is a safe and effective surgical method.

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Incomplete isolation of the left innominate artery from the right aortic arch: a rare case of double-outlet right ventricle with pulmonary stenosis

Cardiology in the Young ◽

10.1017/s1047951119000702 ◽

2019 ◽

Vol 29 (5) ◽

pp. 717-719

Author(s):

Jun Sato ◽

Atsuko Kato ◽

Naoki Ohashi

Keyword(s):

Right Ventricle ◽

Aortic Arch ◽

Rare Case ◽

Surgical Repair ◽

Pulmonary Stenosis ◽

Innominate Artery ◽

Double Outlet Right Ventricle ◽

Right Aortic Arch ◽

Female Infant ◽

The Right

AbstractIsolation of the left innominate artery from the right aortic arch is a rare anomaly. Herein, we present an even rarer case of incomplete isolation of the proximal left innominate artery with the right aortic arch in a 3-month-old female infant with a double-outlet right ventricle and pulmonary stenosis. Surgical repair at 6 months of age was successful, leading to the restoration of adequate flow in the left arm.

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Double Outlet Right Ventricle Associated with Persistent Common Atrioventricular Canal and Pulmonary Stenosis

Cardiology ◽

10.1159/000174001 ◽

1986 ◽

Vol 73 (3) ◽

pp. 160-163

Author(s):

D. Antonelli ◽

G. Mazor ◽

G. Avni

Keyword(s):

Right Ventricle ◽

Pulmonary Stenosis ◽

Double Outlet Right Ventricle ◽

Atrioventricular Canal

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Repair of double outlet right ventricle with doubly-committed ventricular septal defect

Cardiology in the Young ◽

10.1017/s1047951101000531 ◽

2001 ◽

Vol 11 (4) ◽

pp. 415-419 ◽

Cited By ~ 6

Author(s):

Hideki Uemura ◽

Toshikatsu Yagihara ◽

Takayuki Kadohama ◽

Youichi Kawahira ◽

Yoshiro Yoshikawa

Keyword(s):

Ventricular Septal Defect ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Stenosis ◽

Pulmonary Arteries ◽

Ventricular Outflow Tract ◽

Double Outlet Right Ventricle ◽

Left Ventricular ◽

Aortic Orifice ◽

The Right

Objective: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. Methods: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. Results: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117 ± 68 months. Catheterization carried out 16 ± 6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16 ± 7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Conclusion: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

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