Right ventricular “overhaul”—an intermediate step in the biventricular repair of pulmonary atresia with intact ventricular septum

1995 ◽  
Vol 5 (2) ◽  
pp. 161-165 ◽  
Author(s):  
Ash Pawade ◽  
Roger B. B. Mee ◽  
Tom Karl
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Fontan Operation ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intermediate Step ◽  
Intact Ventricular Septum ◽  
Biventricular Repair ◽  
Antegrade Flow ◽  
The Right

SummaryThe management of pulmonary atresia with intact ventricular septum remains controversial. The plan for management at our institution is based on the echocardiographic identification of a well-developed infundibulum. Neonates with such a well-developed infundibulum are prepared for a biventricular repair, whereas those without an infundibulum are groomed for a Fontan operation. In all, 48 neonates with the lesion were admitted to our unit between 1980 and 1992. In 31 neonates with a well-formed infundibulum, the initial palliation consisted mainly of pulmonary valvotomy without cardiopulmonary bypass and construction of a polytetrafluoroethylene shunt from the left subclavian artery to the pulmonary trunk. In most of these patients, growth of the right ventricle could be promoted by establishing antegrade flow through the ventricle. In seven patients in this group, nonetheless, the right ventricle failed to grow satisfactorily. In this subgroup, additional intermediate procedures were performed on the apical component and infundibulum of the ventricle together with the tricuspid and pulmonary valves which could collectively be termed as “right ventricular overhaul.” Five of these patients have gone on to further successful biventricular repairs at a median interval of 24 months after the overhaul. There was no operative mortality in this group. Following the final biventricular repair, there have been no late deaths or reoperations over a total follow-up of 145.4 patient months (mean 22.2 months).

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.

The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results

2007 ◽  
Vol 17 (4) ◽  
pp. 387-396 ◽  
Author(s):  
A. Louise Calder ◽  
Charles R. Peebles ◽  
Christopher J. Occleshaw
Keyword(s):  
Coronary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Coronary Arteries ◽  
Pulmonary Atresia ◽  
Left Ventricular ◽  
Ventricular Septum ◽  
Left Ventricular Myocardium ◽  
Intact Ventricular Septum ◽  
The Right

AbstractBackgroundThe relatively high mortality in patients with pulmonary atresia and intact ventricular septum may be related to the presence of significant coronary arterial anomalies. This retrospective review of cineangiocardiograms was undertaken to further elucidate the types and variety of such coronary arterial abnormalities, and to assess their effect on postoperative survival.Material and resultsDetails regarding coronary arterial anatomy and abnormalities were assessed in 116 patients. We noted the site and severity of lesions, and the presence of fistulous communications from the right ventricle to the coronary arteries, assessing the proportion of left ventricular myocardium affected by coronary arterial interruptions or significant stenoses, in other words, the amount dependent on coronary circulation from the right ventricle. We also measured diameters of the tricuspid and mitral valves. Fistulas were found in 87 patients (75%), interruptions of major coronary arteries in 40 patients (34%), lack of connections between the coronary arteries and the aorta in 18 patients (16%), and single origin of a coronary artery, with the right coronary artery arising from the left, in 6 patients (5%). We found increased mortality in 47 patients (40%) who had a right ventricular-dependent coronary arterial circulation. The presence of fistulas in itself was not associated with higher mortality, but the presence of coronary arterial interruptions (p = 0.05), and a higher myocardial score (p = 0.0009), were.ConclusionWe encountered a higher prevalence of both coronary arterial abnormalities and right ventricular-dependent circulation than previously reported. Awareness of the severity of the coronary arterial abnormalities should assist in planning treatment.

Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?

1992 ◽  
Vol 2 (4) ◽  
pp. 391-394 ◽  
Author(s):  
Carlo Vosa ◽  
Paolo Arciprete ◽  
Giuseppe Caianiello ◽  
Gaetano Palma
Keyword(s):  
Right Ventricular ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Shunt Procedure ◽  
The Right ◽  
Overall Mortality

SummaryBetween February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

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

Risk factors for augmentation of the flow of blood to the lungs in pulmonary atresia with intact ventricular septum after radiofrequency valvotomy

2005 ◽  
Vol 15 (2) ◽  
pp. 141-147 ◽  
Author(s):  
Mazeni Alwi ◽  
Geetha Kandavello ◽  
Kok-Kuan Choo ◽  
Bilkis A. Aziz ◽  
Hasri Samion ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Arterial Duct ◽  
The Right ◽  
Patent Arterial Duct

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract.The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve.We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups.The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of −1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of −0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle.The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

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.

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