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

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.

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951100000214 ◽

1991 ◽

Vol 1 (2) ◽

pp. 105-113 ◽

Cited By ~ 31

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.

Download Full-text

Vascular aneurysm producing divided right atrium in a patient with pulmonary atresia and intact ventricular septum

Cardiology in the Young ◽

10.1017/s1047951100009264 ◽

2000 ◽

Vol 10 (3) ◽

pp. 281-285 ◽

Cited By ~ 1

Author(s):

John C. Wood ◽

Miguel Reyes-Mugica ◽

Gary Kopf

Keyword(s):

Coronary Artery ◽

Coronary Arteries ◽

Right Coronary Artery ◽

Right Atrium ◽

Pulmonary Atresia ◽

Pulmonary Trunk ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Atrioventricular Groove ◽

The Right

AbstractWe describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.

Download Full-text

A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum

Cardiology in the Young ◽

10.1017/s1047951100006429 ◽

2000 ◽

Vol 10 (1) ◽

pp. 60-63 ◽

Cited By ~ 6

Author(s):

Robert M. Freedom ◽

Shi-Joon Yoo ◽

Alexander Javois

Keyword(s):

Left Ventricular Function ◽

Right Ventricular ◽

Coronary Arteries ◽

Coronary Circulation ◽

Pulmonary Atresia ◽

Left Ventricular ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Descending Thoracic Aorta ◽

Collateral Vessel

AbstractA patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951100011744 ◽

1995 ◽

Vol 5 (2) ◽

pp. 161-165 ◽

Cited By ~ 11

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).

Download Full-text

Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure

Cardiology in the Young ◽

10.1017/s1047951104003178 ◽

2004 ◽

Vol 14 (3) ◽

pp. 335-337 ◽

Cited By ~ 7

Author(s):

Saad Khoshhal ◽

George G. S. Sandor ◽

Walter J. Duncan

Keyword(s):

Right Ventricular ◽

Ventricular Dysfunction ◽

Pulmonary Atresia ◽

Ventricular Volume ◽

Left Ventricular ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Ventricular Failure ◽

Severe Left Ventricular Dysfunction ◽

The Right

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.

Download Full-text

Right Ventricular Coiling to Limit Right Ventricle to Coronary Artery Connections in Pulmonary Atresia with Intact Ventricular Septum following Stage 2 Palliation

10.1055/s-0038-1628337 ◽

2018 ◽

Author(s):

J. Hummel ◽

B. Stiller ◽

J. Kroll ◽

M. Siepe ◽

J. Grohmann

Keyword(s):

Coronary Artery ◽

Right Ventricle ◽

Right Ventricular ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Intact Ventricular Septum

Download Full-text

Possible link between right ventricular coronary sinusoids and noncompaction sinusoids in pulmonary atresia with intact ventricular septum patients that later develop left ventricular noncompaction

Medical Hypotheses ◽

10.1016/j.mehy.2014.04.010 ◽

2014 ◽

Vol 83 (1) ◽

pp. 53-55 ◽

Cited By ~ 2

Author(s):

Isa Ozyilmaz ◽

Yakup Ergul ◽

Alper Guzeltas ◽

Ender Odemis

Keyword(s):

Right Ventricular ◽

Pulmonary Atresia ◽

Left Ventricular ◽

Ventricular Septum ◽

Left Ventricular Noncompaction ◽

Intact Ventricular Septum ◽

Ventricular Noncompaction

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951100008003 ◽

1992 ◽

Vol 2 (4) ◽

pp. 391-394 ◽

Cited By ~ 5

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%.

Download Full-text