Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal

2021 ◽  
Vol 12 (2) ◽  
pp. 185-194 ◽  
Author(s):  
Noveen Davidson ◽  
Fiona Doig ◽  
Eliazar Dimpalapang ◽  
John Stirling ◽  
Thomas Gentles ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Treatment Options ◽  
Pulmonary Atresia ◽  
Interventional Treatment ◽  
Coronary Fistula ◽  
Coronary Abnormalities ◽  
Coronary Steal ◽  
The Right ◽  
Late Survival ◽  
Coronary Fistulae

Background: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. Methods: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. Results: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days ( P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. Conclusion: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

scholarly journals Pulmonary atresia with intact septum: Growth of the right ventricle and tricuspid valve following primary procedure

1996 ◽  
Vol 27 (2) ◽  
pp. 343-344
Author(s):  
P.E.F. Daubeney ◽  
Z. Slavìk ◽  
B.R. Keeton ◽  
R.H. Anderson ◽  
S.A. Webber
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Primary Procedure ◽  
The Right

scholarly journals Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum

1991 ◽  
Vol 101 (2) ◽  
pp. 222-229 ◽  
Author(s):  
William G. Williams ◽  
Patricia Burrows ◽  
Robert M. Freedom ◽  
George A. Trusler ◽  
John G. Coles ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
The Right

Balloon Valvuloplasty Through the Right Ventricle: Another Treatment of Pulmonary Atresia With Intact Ventricular Septum

2013 ◽  
Vol 95 (5) ◽  
pp. 1670-1674 ◽  
Author(s):  
Qian-zhen Li ◽  
Hua Cao ◽  
Qiang Chen ◽  
Gui-Can Zhang ◽  
Liang-Wan Chen ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Balloon Valvuloplasty ◽  
Intact Ventricular Septum ◽  
The Right

Pulmonary atresia with intact ventricular septum in the fetus

1992 ◽  
Vol 2 (4) ◽  
pp. 367-376 ◽  
Author(s):  
Lindsey D. Allan ◽  
Andrew Cook
Keyword(s):  
Right Ventricle ◽  
Poor Prognosis ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Fetal Life ◽  
Intact Ventricular Septum ◽  
Cross Sectional ◽  
The One ◽  
The Right ◽  
Echocardiographic Findings

AbstractThe objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.

Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period

1992 ◽  
Vol 2 (4) ◽  
pp. 377-381 ◽  
Author(s):  
Michael L. Rigby ◽  
Micelia Salgado ◽  
Celia Silva
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Cross Sectional ◽  
Mitral Valves ◽  
Significant Difference ◽  
The Right ◽  
Pulmonary Valvar Stenosis

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum

1991 ◽  
Vol 1 (2) ◽  
pp. 105-113 ◽  
Author(s):  
Robert H. Anderson ◽  
Christine Anderson ◽  
James R. Zuberbuhler
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Tricuspid Valve ◽  
Coronary Arteries ◽  
Pulmonary Atresia ◽  
Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Ventricular Cavity ◽  
The Right

SummaryAtresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.

scholarly journals The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa

2009 ◽  
Vol 138 (5) ◽  
pp. 1160-1166 ◽  
Author(s):  
Shu-Chien Huang ◽  
Kozo Ishino ◽  
Shingo Kasahara ◽  
Ko Yoshizumi ◽  
Yasuhiro Kotani ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
The Right
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