Isolated Unilateral Absence of the Pulmonary Artery

Abstract

SUMMARYA 10-month-old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 months after operation due to massive uncontrollable hemoptysis from the right lung.Isolated unilateral absence of the pulmonary artery is a rare lesion. In our review of the world literature as of November 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery have been reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. Our experience with this case has led us to consider a lung biopsy before proceeding to the surgical repair of the lesion. If the affected lung shows arteriovenous abnormalities the operation should not be recommended. Additional Indexing Words: Pulmonary hypertensionPulmonary arteriovenous fistula NILATERAL absence of a pulmonary artery (UAPA) is an uncommon lesion. It is frequently associated with cardiac defects, primarily tetralogy of Fallot. Isolated UAPA is rare. In a review of the world literature

Right pulmonary artery agenesis presenting with uncontrolled asthma in an adult: a case report

“…In a literature review by Ten Harkel et al [2], recurrent pulmonary infections were present in 37% of cases, dyspnea or exercise limitations in 40%, hemoptysis in 20%, and high-altitude pulmonary edema in 12%. Pulmonary hypertension was found in 44% of patients which is higher than a previous report of 20-25% [3,4]. …”

Neonatal diagnosis of isolated absence of the right pulmonary artery: a case report and review of the literature

“…In the other few cases of infant diagnosis of isolated UAPA reported in literature to date, the initial sign was either cyanosis or a murmur. As the patients get older, they may develop a variety of symptoms, including pulmonary hypertension [4]. The incidence of the latter has been reported to range between 19% to 44% [9, 10].…”

“…UAPA is often associated with other cardiac anomalies; however it may seldom occur as an isolated lesion, which mostly concerns the right pulmonary artery [2, 3]. Among the cardiac malformations possibly associated with UAPA, tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia are the most frequent ones [4]. These anomalies are often associated with cyanosis, heart failure or growth retardation in the neonate and/or infant.…”

“…Due to the rarity of the disease, there is no yet general consensus regarding the treatment of UAPA. Therefore, the treatment must be tailored according to the clinical signs and symptoms, the anatomy of pulmonary artery and aortic-pulmonary collaterals, as well as the presence of associated cardiovascular anomalies and pulmonary hypertension [4, 5]. The diagnosis of UAPA soon after birth and/or in early infancy allows for early medical and/or surgical treatment, which in turn improves the effects on symptoms and outcome.…”

Metastatic Non-Small-Cell Lung Cancer in the Setting of Unilateral Agenesis of the Left Pulmonary Artery: A Case Report and Comprehensive Literature Review

“…It was first described in 1868 [4], with 352 cases reported in the literature—237 of which were associated with cardiac anomalies [1, 5]. In the absence of associated cardiac abnormalities, isolated UAPA might remain relatively indolent with delayed diagnosis being made later in adulthood due to symptoms of exertional dyspnea (18-40%), recurrent respiratory infections (37%), pulmonary hypertension (19-44%), and hemoptysis (20%) [6–10]. UAPA is a congenital phenomenon resulting from the involution of the proximal sixth aortic arch with a persistent connection of the intrapulmonary pulmonary artery (PA) with the distal sixth aortic arch [11].…”