Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.

Leval, M de; Bull, Catherine; Stark, Jaroslav; Anderson, Robert H.; Taylor, J F; Macartney, F J

doi:10.1161/01.cir.66.2.272

Cited by 152 publications

(65 citation statements)

References 15 publications

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“…In patients who have tripartite right ventricle 4 , with a patent infundibulum 18 , moderate ventricular hypoplasia at most, and coronary circulation not depending on the right ventricle [6][7][8] , this technique promotes efficient decompression of the ventricular cavity, stimulating its growth 14,19 . Therefore, some potential surgical complications, which may occur in this type of disease, may be avoided.…”

Section: Discussionmentioning

confidence: 99%

“…Its morphological spectrum is broad with cases ranging from extremely hypoplastic tricuspid valves and right ventricles to ventricular cavities of almost normal dimensions [2][3][4] . Ebstein's anomaly of the tricuspid valve and extreme ventricular dilation have also been reported, and they are associated with a poor prognosis 5 .…”

Section: New Percutaneous Techniques For Perforating the Pulmonary Vamentioning

confidence: 99%

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New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

Pedra¹,

Sousa²,

Pedra³

et al. 2001

Arq. Bras. Cardiol.

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Even though pulmonary atresia with intact ventricular septum is an infrequent defect, accounting for less than 1% of congenital heart defects, it is the 3 rd most frequent cyanotic heart defect in the neonatal period 1 . Its morphological spectrum is broad with cases ranging from extremely hypoplastic tricuspid valves and right ventricles to ventricular cavities of almost normal dimensions [2][3][4] . Ebstein's anomaly of the tricuspid valve and extreme ventricular dilation have also been reported, and they are associated with a poor prognosis 5 . In addition, communication between the right ventricular cavity and the coronary arteries is a relatively common finding, which is sometimes associated with coronary circulation partially or totally dependent on the right ventricle [6][7][8] . Due to this anatomic heterogeneity, the therapeutic algorithm should be individualized 6 . The final objective is always to attain biventricular correction with total separation between systemic and pulmonary circulations 6 . However, this is sometimes impossible, and correction with a 1½ ventricle or of the univentricular type (Fontan) is necessary [6][7][8][9] . Cardiac transplantation should also be considered for treating cases with severe stenoses or multiple interruptions in the coronary arteries and secondary left ventricular dysfunction 6,8 . The initial therapeutic approach in the neonatal period should, whenever possible (if the coronary circulation pattern allows), open the pulmonary valve to decompress the right ventricle and stimulate its growth 3,[6][7][8] . During the last decade, perforation of the pulmonary valve to establish continuity between the right ventricle and the pulmonary artery with the aid of interventional catheterization became a reality [10][11][12][13][14][15] , even in Brazil 16. We report 2 percutaneous techniques of valve perforation, which were recently introduced into clinical practice, and their advantages and disadvantages are discussed. Case reportWe report the cases of 3 patients referred to our service from other neonatal units for investigation or treatment of cyanotic congenital heart defects. The clinical, echocardiographic, and hemodynamic data, are listed in tables I, II, and III. It is worth noting that patient 2 had a previous diagnosis of critical pulmonary stenosis. All patients had mild to moderate cyanosis under continuous infusion of prostaglandin; on auscultation, the 2 nd cardiac sound was single and low, and was followed by a mild systolic murmur in the dorsum. On chest X-ray, the cardiac silhouette was slightly enlarged, mainly because of the right atrium, and the pulmonary flow was reduced. The electrocardiogram showed sinus rhythm and left ventricular hypertrophy in all patients. QRS axes ranged from 90º to 120º. In regard to the echocardiographic findings, all patients had situs solitus, pulmonary atresia with imperforate pulmonary valve, and intact ventricular septum. The right ventricle was hy-

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Section: Discussionmentioning

confidence: 99%

Section: New Percutaneous Techniques For Perforating the Pulmonary Vamentioning

confidence: 99%

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

Pedra¹,

Sousa²,

Pedra³

et al. 2001

Arq. Bras. Cardiol.

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“…These results suggested that the growth of the right ventricle was commensurate with the physical growth of the patients, and these results are consistent with those in previous reports. 1,3,16,21 Hanley and colleagues' indicated in their prospective multiinstitutional study that the Z-value of the tricuspid valve did not change during the follow-up period of the patients whose right ventricle-to-pulmonary artery continuity had been established at the initial palliation. Similar to our findings, their study also suggested that the diameter of the tricuspid valve enlarged commensurate with the physical growth of the patients whose right ventricular outflow tract had been initially reconstructed.…”

Section: Discussionmentioning

confidence: 99%

Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum

Mishima

Asano

Sasaki

et al. 2000

Jpn J Thorac Caridovasc Surg

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“…Nevertheless, concerns persist regarding application of Simpson's rule to these small and often distorted ventricles. 12 The RVI described in the present report is a modification of the morphologic measurements of Zuberbuhler and Anderson!. These authors related measurements of the TVA, RV inlet and RV outlet to their left ventricular counterparts in order to obtain a meaningful comparison of pathologic specimens.…”

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confidence: 93%

Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy.

Lewis¹,

Wells²,

Lindesmith³

1983

Circulation

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SUMMARY The initial surgical approach to the infant with pulmonary atresia and intact ventricular (PA-IVS) is to establish an adequate source of pulmonary blood flow and, when possible, relieve right ventricular (RV) outflow obstruction. The selection of patients for pulmonary valvotomy, alone or in combination with a systemic-pulmonary arterial shunt, depends on the presence of an RVoutflow tract and the adequacy of the RV chamber. To evaluate the size of the RV cavity in PA-IVS, an RV index (RVI) was developed using biplane angiographic measurements of the sum of the tricuspid valve annulus and the RV inflow and the RV outflow tracts. The RVI was normalized by relating it to the aortic diameter (Ao) at the diaphragm (RVI/Ao). The RVI/Ao was 13.5 + 1.4 in 20 control subjects and only 7.3 + 2.6 in 26 PA-IVS patients (p < 0.001), and was within the normal range in only two of the 26. Since 1976, pulmonary valvotomy plus a Blalock-Taussig shunt has been performed in 10 infants, with one death. Serial cardiac catheterizations in five of nine survivors demonstrated substantial RV growth in all, with the RVI/Ao increasing from an average of 8.0 to 12.5. In contrast, patients who underwent a shunt alone had no change in RV cavity size. We conclude that pulmonary valvotomy may be performed successfully in most PA-IVS patients, but usually must be combined with a systemic-pulmonary shunt. In a small minority of patients, a normal RV cavity, as evidenced by an RVI/Ao 11, appears to be sufficient to sustain adequate pulmonary blood flow after valvotomy alone. The RVI/Ao ratio is a simple method of quantitatively evaluating RV cavity size and is helpful in planning the initial surgical approach for these infants.THE SURVIVAL of neonates with pulmonary atresia and intact ventricular septum (PA-IVS) is dependent upon the provision of a source of adequate pulmonary blood flow. The use of prostaglandin E, (PGE,) is a valuable, although usually short-term, method for maintaining postnatal patency of the ductus arteriosus (PDA).1-' Therefore, early surgical intervention is necessary to ensure a more permanent route for pulmonary flow. Most surgical approaches to the problem have been based on the pathologic classification of Greenwold et al. ,4' 5 which divides the right ventricle into two anatomic types: a predominant group with a small right ventricular (RV) cavity (type I) and a less common group with a normal-sized or large RV cavity (type II). Accordingly, early reports of the surgical management of these infants emphasized the need for a systemic arterial-pulmonary arterial shunt in the majority of patients with tiny right ventricles.6' 7 Morphologic studies of PA-IVS have documented both a broad spectrum of RV cavity size' and the ability of the right ventricle to increase after successful pulmonary valvotomy.9 Nevertheless, numerous reports have revealed high mortality rates in patients with small right ventricles in whom pulmonary valvotomy was the sole initial surgical procedure.10-3 Therefore, preoperative angiographi...

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Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.

Cited by 152 publications

References 15 publications

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum

Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy.

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