Ann Thorac Surg 2002;73:1322-1324
© 2002 The Society of Thoracic Surgeons
Case report
Pulmonary atresia with intact ventricular septum and systemic-pulmonary collateral arteries
Sonia B. Albanese, MD*b,
Adriano Carotti, MDb,
Alessandra Toscano, MDa,
Bruno Marino, MDa,
Roberto M. Di Donato, MDb
a Department of Pediatric Cardiology, Bambino Gesu Hospital, Rome, Italy
b Department of Cardiac Surgery, Bambino Gesù Hospital, Rome, Italy
Accepted for publication August 24, 2001.
* Address reprint requests to Dr Albanese, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Hospital, Piazza S. Onofrio, 4, 00165 Rome, Italy
e-mail: albanese{at}opbg.net
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Abstract
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Pulmonary atresia with intact ventricular septum and major systemic-pulmonary collateral arteries is a very rare congenital heart lesion with dismal natural history. Herein we report on a case of pulmonary atresia with intact ventricular septum with hypoplastic right ventricle, very small confluent pulmonary arteries, absent arterial duct, and pulmonary blood flow exclusively provided by bronchial-type systemic-pulmonary collateral arteries that was successfully treated at our institution.
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Introduction
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Pulmonary artesia with intact ventricular septum is a heterogeneous congenital heart lesion characterized by a wide variability in size of tricuspid valve and right ventricle (RV) and by abnormalities of coronary arteries. In contrast, there is usually uniformity in the anatomy of central pulmonary arteries (PAs), almost always normal-sized, and in the source of pulmonary blood flow, generally a left-sided arterial duct. Therefore, the prevalence of systemic-pulmonary collateral arteries (SCAs) is extremely low [1–3] compared with that in pulmonary atresia with ventricular septal defect [4]. Herein we report on a case of pulmonary atresia with intact ventricular septum with hypoplastic RV, very small confluent PAs, absent arterial duct, and pulmonary blood flow exclusively provided by SCAs.
A 1-day-old neonate was transferred to our unit for marked cyanosis. On physical examination he had a 2/6 continuous murmur at the left sternal edge. Transcutaneous oxygen saturation was 75%. Two-dimensional echocardiographic and color Doppler examination showed pulmonary atresia with intact ventricular septum with a hypoplastic RV made only of an inlet and a virtual outlet portion. The tricuspid valve z-score was -10.9. There were sinusoids between the RV cavity and the coronary arteries. Neither well-developed PAs nor a patent arterial duct could be detected. Cardiac catheterization showed a reductive RV cavity in communication with the coronary arteries and the aortic root by means of multiple sinusoids. There was no evidence of RV-dependent coronary circulation. The arterial duct was absent. Instead, there were multiple slender SCAs arising from the thoracic aorta and providing retrograde filling of very small confluent PAs (pulmonary artery index = 20 mm2/m2) (Fig 1).
None of the collaterals supplied exclusively any pulmonary district, whereas the PAs, although very small, distributed to the whole lung parenchyma.
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Fig 1. Preoperative angiography. Stop-flow of descending aorta (DAO) showing retrograde filling of hypoplastic native right (RPA) and left (LPA) pulmonary arteries through bronchial-type systemic-pulmonary collateral arteries (*).
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On the basis of this anatomy, an initial surgical strategy of central aortopulmonary shunting was undertaken [5]. Through a midline sternotomy, the main pulmonary trunk and central tiny PAs were dissected free. No remnant of arterial duct was found. Under mild heparin coverage (1 mg/kg), the PAs were clip-occluded, and the main pulmonary trunk was transected just above the atretic pulmonary valve. A 2.7-mm punch-hole was made on the posterior wall of the ascending aorta. Direct end-to-side anastomosis of the main pulmonary artery to the ascending aorta was then performed using a running suture of 7-0 absorbable monofilament. Early postoperative period was complicated by temporary severe arterial desaturation, successfully treated by a 24-hour course of full ventilation with halothane added to the inspired gas mixture. The patient was extubated on the second postoperative day and discharged home on the 10th postoperative day with a transcutaneous oxygen saturation of 85%.
At 6 months of age, the child underwent a control cardiac catheterization that showed satisfactory growth of both central and peripheral PAs, stenosis at the take-off of both PAs from the ascending aorta, and regression of the systemic-to-pulmonary collateral circulation (Fig 2).
The tricuspid valve z-score had also increased to -1.6.
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Fig 2. Postoperative angiography. Angiogram of ascending aorta (AAO) showing stenosis of takeoff of both right (RPA) and left (LPA) pulmonary arteries and satisfactory growth of their central and peripheral portion.
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Two months later an uncomplicated procedure of detachment of pulmonary bifurcation from the aorta, patch reconstruction of pulmonary arterial confluence, and bidirectional cavopulmonary anastomosis was performed. Postoperative follow-up visits were conducted every other month for 8 months with routine two-dimensional echocardiographic investigations that showed a well-functioning cavopulmonary anastomosis and normally sized central PAs.
To date, the child is 16 months old and is awaiting a new cardiac catheterization to guide the next surgical step (total cavopulmonary connection versus one-and-half ventricle repair).
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Comment
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The occurrence of SCAs in patients with pulmonary atresia with intact ventricular septum is extremely rare and usually correlates with major abnormalities of the pulmonary arterial tree [1–3]. In these cases, generally affected by a poor surgical prognosis [2], the occurrence of major SCAs, mostly type 2 or type 3 collateral arteries according to classification by Rabinovitch and associates [4], has been reported both in the presence and in the absence of pulmonary arterial confluence. Our case is characterized by a unique anatomic situation in which normally branched, although severely hypoplastic, confluent PAs coexisted with bronchial-type (Rabinovitch’s type 1) collateral arteries, providing the whole pulmonary blood perfusion. In fact, no remnant of arterial duct was found.
According to De Ruiter and colleagues [6], the occurrence of pulmonary obstruction during late fetal gestation may induce development of bronchial arteries into SCAs (Rabinovitch’s type 1). Those collateral arteries are typically anastomosed to the pulmonary arterial tree at the intraacinar arteriolar level [4]. Consequently, they cannot maintain long-term patency of both intrapulmonary branching and central portion of the PAs in the absence of forward flow through the RV outflow tract or the arterial duct. The peculiar anatomy of the lung vasculature in the case reported could be explained with the absence of in utero development of the arterial duct. The patency of the PAs would have been maintained by the antegrade flow through the RV outflow tract, patent until late fetal gestation, whereas the degree of pulmonary arterial hypoplasia would reflect the interval between occurrence of prenatal pulmonary atresia and postnatal diagnosis.
The initial surgical procedure was chosen to stimulate the growth of the PAs under pressure stress [5]. Unlike in the case of pulmonary atresia with ventricular septal defect, however, the option of a central shunting procedure rather than a palliative RV outflow tract reconstruction was mandatory owing to the restrictive size of the RV cavity, the presence of sinusoids, and the absence of a ventricular septal defect. The effective growth of the pulmonary arterial tree, as seen at the postoperative cardiac catheterization, sustains the validity of this approach. In the future for our patient, pending a new hemodynamic investigation, a decision will have to be made about his suitability for one-and-half ventricle versus Fontan arrangement.
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References
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Abstract
Introduction
