Neonatal Pneumonectomy for Isolated Unilateral Pulmonary Artery Agenesis Charles C. Canver, MD, John D. Pigott, MD, and Robert M. Mentzer, Jr, MD Division of Cardiothoracic Surgery, Department of Surgery, State University of New York at Buffalo School of Medicine and Biomedical Sciences, Buffalo, New York

A 23-day-old female newborn was evaluated for acyanotic respiratory distress immediately after birth. Echocardiogram and cardiac catheterization revealed absence of the right pulmonary artery. Subsequent course was complicated by necrotizing bronchopneumonia. Despite antibiotic therapy and ventilator support she failed


solated unilateral pulmonary artery agenesis without associated cardiac anomalies is rare. Although fewer than 60 cases have been reported [lJ, a majority were first seen in adulthood as either an incidental finding on routine chest roentgenogram or a result of symptoms related to complications [2, 31. The malformation is characterized by unilateral absence of a connection between the main pulmonary artery and the parenchymal pulmonary vessels. No specific surgical treatment is considered absolutely reparative. Operation is indicated primarily to treat complications, eg, hemoptysis or bronchiectasis. In this report, a case of isolated unilateral absence of the right pulmonary artery requiring pneumonectomy for necrotizing pneumonia is reported. A 23-day-old female newborn was transferred to the intensive care nursery of the Children's Hospital a t the State University of New York at Buffalo immediately after birth because of persistent peripheral cyanosis and intercostal retractions. The newborn was full term (4,000 g) and the product of an uncomplicated vaginal delivery. The mother's prenatal course was uneventful. On admission, physical examination revealed a temperature of 37.1"C; pulse of 150 beatdmin, respiration rate of 28/min, and blood pressure of 90140 mm Hg. Auscultation of the chest revealed diminished breath sounds on the right. The heart rate was regular without murmurs, and the abdomen was unremarkable. Laboratory studies revealed a hemoglobin level of 133 g1L (13.3 g1dL) and a white blood cell count of 2.01 x 107/L (20,100imL). The chemistry profile was normal with the exception of an elevated total bilirubin level 113 pmoliL (6.6 mg1dL); arterial blood gases revealed a p H of 7.42, carbon dioxide tension of 38 mm Hg, and oxygen tension of 95 mm Hg on 80% supplemental hood-oxygen. The electrocardiogram showed right axis deviation and right ventricular hypertrophy. Chest roentgenogram revealed normal size heart but diminished vascular markings (Fig 1). Echocardiogram demonstrated absence of the right pulmonary artery. Accepted for publication Dec 31, 1990. Address reprint requests to Dr Canver, Department of Surgery, State Universitv of New York at Buffalo, 462 Grider St, Buffalo, NY 14215.

6 1991 by The Society of Thoracic Surgeons

to improve; right pneumonectomy was performed to remove the source of sepsis. This case represents an example in which an infectious complication in a rare congenital pulmonary malformation served as an indication for neonatal pneumonectomy. (A1717 Tliornc Surg 1991;52:294-5) On the third day of life, increasing respiratory distress developed and the neonate required ventilatory support. A dynamic computed tomographic scan showed cystic changes throughout the entire right lung field. A nuclear scan revealed absence of both right lung ventilation and perfusion. O n the 14th day, increasing secretions, marked leukocytosis, and right parenchymal lung consolidation developed. Aspirates from the endotracheal tube grew Staplylococc-us nureus. Antibiotic treatment consisted of vancomycin, gentamicin, and ampicillin intravenously. Because the patient failed to improve with supportive therapy and was ventilator dependent, cardiac catheterization was performed to confirm absence of other cardiac anomalies. The catheterization demonstrated absence of a right pulmonary artery and only a patent foramen ovale (Fig 2). The pulmonary arterial pressure was 50115 mm Hg; the right ventricular pressure was 5016 mm Hg. To control subsequent sepsis, the patient underwent a right pneumonectomy. Intraoperatively a small pulmonary artery containing gelatinous material precluded revascularization. The lung itself was unilobar, containing a large necrotic cavity. The postoperative recovery was uneventful and the patient was discharged 9 days after operation. A roentgenogram 1 year later demonstrated hyperexpansion of the left lung with marked shift of the mediastinum and heart (Fig 3). At present she is healthy and growing normally.

Comment Isolated unilateral absence of a pulmonary artery was first described by Fraentzel in 1868 [4]. The pathogenesis of this malformation and associated hypoplastic lung is unknown. It appears that agenesis or underdevelopment of the sixth branchial arch during the embryonic life contributes to the development of this anomaly. Usually pulmonary hypoplasia is present on the affected side and systemic bronchial arteries persist as the only arterial supply. The pulmonary veins enter the left atrium normally. The involved pulmonary artery is usually on the side opposite the aortic arch. When the left side is involved, associated cardiac anomalies such as tetralogy of Fallot are common. 0003-4975/911$3.50



Ann Thorac Surg 1991;52:29&5

Fig 1 . Preoperative rurntpwograiri illiistrntcs abscricc of right plrironary vascular rnarkings.

The clinical presentation of patients with this rare entity is variable. Some reports indicate that 30% of patients may be asymptomatic throughout life [ 5 ] . The diagnosis may be suggested by the chest roentgenogram. Less than 10% of the patients have hemoptysis; rarely, there is a history of recurrent pulmonary infection, chest pain, dyspnea on exertion, cyanosis, or congestive heart failure. Findings on physical examination are generally unremarkable with the exception of decreased breath sounds on the involved side. The diagnosis is confirmed by pulmonary arterial angiography and nuclear scanning. One of the most important complications associated with this malformation is the development of pulmonary hypertension [6]. Individuals in whom pulmonary hypertension develops


Fig 3 . Chest roentpiograni 2 year after pneurnonectotny zoifh shift of the licart to right of niidline.

without cardiac anomalies usually die at a young age and succumb to right-sided heart failure. Interestingly, patients with unilateral absence of right pulmonary artery are reported to be particularly susceptible to high-altitude pulmonary edema [7]. Usually operation is not indicated for isolated absent pulmonary artery because the symptoms are mild or absent. If surgical intervention is deemed necessary because of recurrent hemoptysis or pneumonia, the options include revascularization or lung resection [ 8 ] . Our case demonstrates that pneumonectomy in the neonate is feasible and may be the only alternative in treating certain complications associated with this malformation.

References 1. Werber J, Ramilo JL, London R, Harris VJ. Unilateral absence of a pulmonary artery. Chest 1983;84:729-32. 2. Mehta AC, Ahmad A, Golish JA, Buonocore E. Congenital anomalies of the lung in the adult. Cleve Clin Q 1983;SO: 401-16. 3 . Luck SR, Reynolds M, Raffensperger JG. Congenital bronchopulmonary malformations. Curr Probl Surg 1986;23:24&314. 4. Fraentzel 0. Ein Fall von abnormer Communication der Aorta mit der Arteria pulmonalis. Virchows Arch Pathol Anat 1868; 43:420-6. 5. Bahler RC, Carson P, Traks E, Levene A, Gillespie D. Absent right pulmonary artery. Problems in diagnosis and management. Am J Med 1969;46:64-71. 6 . Pool PE, Vogel JHK, Blount SG. Congenital unilateral absence of a pulmonary artery: the importance of flow in pulmonary hypertension. Am J Cardiol 1962;10:70&32. 7. Hackett P, Creagh CE, Grover RF, et al. High altitude pulmonary edema in persons without the right pulmonary artery. N Engl J Med 1980;302:1070-3. 8. Maier H. Absence or hypoplasia of a pulmonary artery with anomalous systemic artery to the lung. J Thorac Surg 1954;28: 14542.

Neonatal pneumonectomy for isolated unilateral pulmonary artery agenesis.

A 23-day-old female newborn was evaluated for acyanotic respiratory distress immediately after birth. Echocardiogram and cardiac catheterization revea...
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