Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis

2019 ◽  
Vol 10 (2) ◽  
pp. 157-163 ◽  
Author(s):  
Evelyn S. Huang ◽  
Jeremy L. Herrmann ◽  
Mark D. Rodefeld ◽  
Mark W. Turrentine ◽  
John W. Brown
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Cardiac Transplantation ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Optimal Choice ◽  
Term Survival ◽  
Clinical Records ◽  
Rastelli Operation

Objectives: Our preferred approach for the surgical treatment of patients with D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis has been the Rastelli operation. We herein evaluate our 30-year experience with this procedure. Methods: Clinical records for patients who underwent the Rastelli operation between 1988 and 2017 at our institution were retrospectively reviewed. Primary outcomes included freedom from death or cardiac transplantation and freedom from conduit reintervention. Results: Forty-seven patients met inclusion criteria. Mean follow-up was 11.7 ± 6.8 years. Forty-three (91.5%) patients received a palliative systemic-to-pulmonary artery shunt and/or atrial septostomy prior to the Rastelli procedure. Five (10.6%) patients required ventricular septal defect enlargement at the time of the Rastelli procedure. The overall mean right ventricle-to-pulmonary artery conduit size was 17.0 mm. Mortalities included one early and three late deaths. Freedom from death or cardiac transplantation was 93% and 84% at 5 and 25 years, respectively. Seven patients required pacemaker placement, two immediately postoperatively and five late. Freedom from conduit replacement was 85% and 25% at 5 and 15 years, respectively. Seven (14.9%) patients required a second conduit intervention. Forty-one (87.2%) patients were New York Heart Association class I or II at the most recent follow-up. Conclusions: The Rastelli operation for D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis offers excellent mid- to long-term survival. The need for conduit replacement remains the most common indication for reintervention, and further study of the optimal choice of conduit will be useful.

Double aortic arch: an elusive diagnosis in a child with CHD

2021 ◽  
pp. 1-3
Author(s):  
Mariana Lemos ◽  
Miguel Fogaça da Mata ◽  
Ana Coutinho Santos
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Aortic Arch ◽  
Ct Angiography ◽  
Septal Defect ◽  
Vascular Ring ◽  
Pulmonary Atresia ◽  
Double Aortic Arch ◽  
Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

Rastelli Operation for Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis

2011 ◽  
Vol 91 (1) ◽  
pp. 188-194 ◽  
Author(s):  
John W. Brown ◽  
Mark Ruzmetov ◽  
Daniel Huynh ◽  
Mark D. Rodefeld ◽  
Mark W. Turrentine ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Rastelli Operation

Coarctation of the Left pulmonary artery associated with congenitally corrected transposition

1999 ◽  
Vol 9 (2) ◽  
pp. 207-209 ◽  
Author(s):  
Martial M. Massin ◽  
Götz von Bernuth
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Left Pulmonary Artery ◽  
First Report ◽  
Ductal Tissue ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

AbstractWe describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.

scholarly journals Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Julia Illner ◽  
Holger Reinecke ◽  
Helmut Baumgartner ◽  
Gerrit Kaleschke
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Bare Metal

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

scholarly journals Long-Term Follow-Up of the Conal Flap Method for Tricuspid Malinsertion in Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis

2016 ◽  
Vol 102 (1) ◽  
pp. 186-191
Author(s):  
Tai Fuchigami ◽  
Mitsugi Nagashima ◽  
Takeshi Hiramatsu ◽  
Goki Matsumura ◽  
Minori Tateishi ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Long Term Follow Up

Abstract 12635: Nationwide Long-term Follow-up of Pulmonary Atresia With Ventricular Septal Defect

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Anu Kaskinen ◽  
Juha-Matti Happonen ◽  
Ilkka P Mattila ◽  
Olli M Pitkänen
Keyword(s):  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Palliative Surgery ◽  
Total Study Population ◽  
Collateral Arteries

Introduction: The naturally poor survival of pulmonary atresia with ventricular septal defect (PA+VSD) has improved due to evolved perioperative and surgical treatment. Studies including PA+VSD patients, both with and without major aortopulmonary collateral arteries (MAPCAs), with extensive follow-up are scarce. This nationwide study aimed to investigate survival and surgical treatment in PA+VSD patients with and without MAPCAs. Methods: Study comprised 109 PA+VSD patients born in Finland between 1970 and 2007. We reviewed retrospectively medical records and operative reports through December 2011, as well as first available angiograms and preoperative angiograms prior to repair attempt. Results: The median follow-up time for the total study population, including also patients who died during the follow-up, was 11.4 years (IQR 0.8 - 21.1). The incidence of PA+VSD, which could be determined reliably from 1995 to 2007, was 6.1 per 100 000 live births. Although the patients with (n = 43) or without MAPCAs (n = 66) showed no difference in survival (p = 0.74), the patients without MAPCAs had better probability to achieve repair (64% vs. 28%, p < 0.0001). The bigger size of true central pulmonary arteries assessed by McGoon index at first angiogram [HR 0.66 (CI95% 0.49 - 0.88) per 0.5 McGoon index units, p = 0.006] and achievement of repair [HR 0.07 (CI95% 0.03 - 0.17), p < 0.0001] improved the overall survival. After successful repair survival was 93% at 1 year and 91% from 2 years on. Palliated patients, instead, had survival at 1, 5, 10, and 20 years of age of 55%, 42%, 34%, and 20% respectively. However, patients with right ventricle - pulmonary artery connection and septal fenestration had better survival than rest of the palliated patients (p = 0.001). Palliation with a systemic-pulmonary artery shunt increased McGoon index by 41% (p < 0.0001). Conclusions: The patients with MAPCAs had higher risk to remain palliated than patients without, although their survival was similar. Survival of PA+VSD was influenced by the initial size of true central pulmonary arteries and whether repair was achieved. Although palliative procedures may not improve the final outcome of PA+VSD, palliative surgery may have a role in its treatment.

CORRECTED TRANSPOSITION OF THE GREAT VESSELS OF THE HEART

PEDIATRICS ◽  
1957 ◽  
Vol 20 (4) ◽  
pp. 626-646
Author(s):  
Ray C. Anderson ◽  
C. Walton Lillehei ◽  
Richard G. Lester
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Laboratory Findings ◽  
Left Border ◽  
Great Vessels ◽  
Corrected Transposition

Corrected transposition of the great vessels is described, together with the probable embryologic basis for the defect. This defect has assumed great importance because of the necessity of recognizing its presence before surgical exploration for correction of the frequently associated ventricular septal defects and pulmonary stenosis. The presence of corrected transposition interferes with the surgical approach because of the anomalous coronary pattern and the inverted location of the defects. The physical and laboratory findings in 17 patients are tabulated and discussed. These included six with a ventricular septal defect, three with pulmonary stenosis, one with a ventricular and an atrial septal defect, one with a ventricular septal defect and left-sided atrioventricular valve stenosis, one with a ventricular septal defect and pulmonary stenosis, two with a reversing patent ductus arteriosus, and three with a ventricular septal defect and a small leftsided ventricle. The electrocardiogram usually shows A-V block, most often first degree, or A-V dissociation, inverted QRS patterns in the precordial leads (qR in V1 and RS in V6), peaked P waves in lead 2, widened QRS complexes, and upright T waves in the precordial leads, beginning either in RV4 or V1. Roentgenograms may demonstrate an unusual appearance of the upper left border of the heart. The main pulmonary artery may deeply indent the barium-filled esophagus, and the left pulmonary artery may be noted to be medially placed. The diagnosis can be definitely established by angiocardiography in the anteriorposterior view. The main pulmonary artery lies medially, and the aorta arises from the upper left border of the heart. Also diagnostic is the anomalous and difficult course taken by the cardiac catheter in entering the medially-placed pulmonary artery. This defect should be suspected in all patients where the pulmonary artery cannot be entered at cardiac catheterization. If pulmonary stenosis is present, the second sound below the left clavicle will not be as soft as usually noted with this defect. Certain considerations deemed of value to the surgical management of the associated intracardiac defects occurring in this series of patients with corrected transposition are presented.

Single versus Biventricular Repair for Discordant Hearts

1996 ◽  
Vol 4 (3) ◽  
pp. 161-163 ◽  
Author(s):  
KG Jaya Prasanna ◽  
Rajesh Sharma ◽  
Krishna Subramany Iyer ◽  
Balram Airan ◽  
Anil Bhan ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Early Death ◽  
Biventricular Repair ◽  
Long Term Result ◽  
Ventricular Septal Defect Closure ◽  
Double Switch

We reviewed our surgical experience, over a 7-year period, of 38 patients with congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis, who were anatomically well-suited for a biventricular repair. Follow-up ranged from 2 to 9 years (mean 5.3 years). One group of patients underwent a univentricular repair; there were 2 early deaths (8%) among the 24 patients who underwent a Fontan-type repair and 5 patients had prolonged pleural effusion. There was no early mortality in the 3 patients who underwent a bidirectional Glenn anastomosis but there was 1 late death. Patients undergoing a biventricular repair comprised 6 who had closure of a ventricular septal defect and pulmonary valvotomy, and 5 who had ventricular septal defect closure and conduit repair. There was 1 early death (9%) and 2 patients developed iatrogenic complete heart block in this group but there was no late mortality. None of these patients had a double switch procedure. With the advent of the double switch procedure, there are now 3 modes of management for these defects. Determining which of these provides the best long-term result is still a matter for debate.

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