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Unilateral pulmonary artery atresia in an adult: A case report.

ABSTRACT: IntroUnilateral pulmonary artery atresia (UPAA), while encountered frequently in the congenital cardiac anomaly cohort, is occasionally diagnosed in adulthood after typical symptoms of hemoptysis, pulmonary infection, or as an incidental finding on contrast CT scan. Due to its rarity, a brief discussion of UPAA and its treatment is warranted.Case reportA 35 year old male presented with three days of hemoptysis. After diagnosis of right UPAA, he underwent angioembolization of 6 large systemic collaterals supplying his right lung, followed by right pneumonectomy. He was discharged on post-operative day 3, and at follow up 6 weeks later was doing well with minimal residual incisional pain and excellent pulmonary reserve.ConclusionsUPAA presents classically with hemoptysis, but also with pneumonia, pulmonary hypertension, or incidentally. Management includes selective collateral embolization, pneumonectomy, or medical management directed towards decreasing pulmonary hypertension in patients unable to tolerate pneumonectomy due to comorbidities. Pneumonectomy in these patients is characterized by dense and hypervascular adhesions, with large volume blood loss expected during adhesiolysis, which can be decreased with pre-operative embolization. Outcomes are typically excellent in otherwise healthy patients.

SUBMITTER: Smith NE

PROVIDER: S-EPMC6297891 | biostudies-other | 2019

REPOSITORIES: biostudies-other

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Project description:RationaleHemoptysis is an uncommon chief complaint but a distressing symptom in pediatric patients. Due to the recurrence and mortality in minor patients, an accurate diagnosis of the underlying cause is quite essential for treatment. The etiologies causing hemoptysis in children are similar to that in adults. Isolated unilateral pulmonary vein atresia (PVA), as an unusual cardiovascular anomaly, has rarely been reported to be an etiology of hemoptysis in children.Patient concernsA 2-year and 11-month-old boy was admitted into our hospital with a complaint of recurrent hemoptysis for 2 months and the symptom became more aggravated in recent 4 days before admission. Physical examination was only remarkable for slightly diminished breath sounds over the left lung field, pale face, and colorless lip. Series of targeted laboratory evaluation were negative expect for anemia. Due to the identification of asymmetrical transparency of bilateral lung, slight emphysema of right lung, less volume of left lung with ground-glass opacity and reticular opacity, and ipsilateral mediastinal shift on chest CT, and varices of submucosal vessels in the left bronchial tree on the fiber-optic bronchoscope.DiagnosesIt more likely indicated a congenital cardiovascular disease. The diagnosis of left isolated unilateral PVA was ultimately confirmed through chest CT angiography (CTA) with three-dimensional (3D) reconstruction.InterventionsSince the boy did not complain with hemoptysis after admission, respiratory tract infections seldom occurred and no pulmonary hypertension was detected, a conservative approach was chosen with periodic clinical follow-up after discussing with the cardiac surgeons and in accordance to his parents' own wishes.OutcomesFortunately, he was doing well after 3 months of clinical observation.LessonsWe firstly reported a rare case of hemoptysis in children secondary to isolated unilateral PVA with no associated congenital heart disease in Chinese population. It is significant to improve the recognition and prompt diagnosis of this rare condition for pediatric clinicians, and widen the etiology spectrum of hemoptysis in children. The diagnosis of unilateral PVA should be considered for a patient with recurrent hemoptysis and imaging findings that indicate hypoplastic lung, ipsilateral mediastinal shift, and smooth margins of left atrium without evidence of rudimentary pulmonary veins.

Project description:Background?Aorta-pulmonary (A-P) artery fistula following a stab wound to the chest with superimposed infective endocarditis (IE) is a rare, often unrecognized presentation. Herein, we report a case of A-P fistula due to stab chest assessed by two- and three-dimensional (3D) imaging.Case summary?A 30-year-old man presented with a history of being stabbed in the chest with a screwdriver. The chest wall laceration was sutured, an intercostal drain inserted for a haemopneumothorax, and he was subsequently discharged. He presented 3?weeks later with exertional dyspnoea, fever, rigours, and loss of weight. On examination, he had a wide pulse pressure and a harsh continuous murmur in the 2nd left intercostal space associated with a palpable thrill. Blood tests revealed raised infective markers and anaemia. All blood cultures were sterile. On echocardiography, the aortic and pulmonary valve was severely damaged, with suspicion of superimposed vegetations secondary to IE. There was severe aortic and pulmonary valve regurgitation. A fistulous connection was noted between the aorta and main pulmonary artery, just below the commissure adjoining the right and left coronary sinus of the aortic valve. On 3D imaging, the defect was quantified. The patient was subsequently referred for aortic and pulmonary valve replacement and closure of the A-P fistula. The presence of multiple vegetations was confirmed intraoperatively. He also received a 6-week course of intravenous antibiotics.Discussion?We have described a rare case of an A-P fistula due to a stab wound to the chest complicated by IE. In a patient with stab wound to the chest, a high index of suspicion of cardiac involvement must be maintained, and a careful search for intracardiac shunts must be made on echocardiography, prior to discharge. Furthermore, in addition to two-dimensional imaging, 3D imaging proved useful in providing a comprehensive assessment of the morphology of the lesion prior to surgery.

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Unilateral pulmonary artery atresia in an adult: A case report.

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