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Right-side pulmonary agenesis with atrial septal defect in adult.

ABSTRACT: Pulmonary agenesis, a rare congenital condition, is incompatible with life when present bilateral, while unilateral agenesis is usually detected in infancy or early childhood. Rare asymptomatic patients may reach adulthood undiagnosed, with signs mimicking common conditions presenting as radiopaque hemithorax with ipsilateral mediastinal shift. Here, we describe a case of a young lady, with history of consanguinity, who presented with complaints, suggestive of lower respiratory tract infection, and was investigated and diagnosed to be a case of right-side pulmonary agenesis with large ostium secondum atrial septal defect. Our present case emphasizes the importance of presence of pulmonary agenesis with cardiac congenital anomaly, remaining asymptomatic until adulthood, particularly in patients born of parents with consanguineous marriages.

SUBMITTER: Das Shukla A

PROVIDER: S-EPMC4818584 | biostudies-other | 2016 Apr

REPOSITORIES: biostudies-other

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Project description:Background Despite correction of the atrial septal defect (ASD), patients experience atrial fibrillation frequently and have increased morbidity and mortality. We examined physical capacity, cardiac performance, and invasive hemodynamics in patients with corrected ASD. Methods and Results Thirty-eight corrected patients with isolated secundum ASD and 19 age-matched healthy controls underwent right heart catheterization at rest and during exercise with simultaneous expired gas assessment and echocardiography. Maximum oxygen uptake was comparable between groups (ASD 32.7±7.7 mL O2/kg per minute, controls 35.2±7.5 mL O2/kg per minute, P=0.3), as was cardiac index at both rest and peak exercise. In contrast, pulmonary artery wedge v wave pressures were increased at rest and peak exercise (rest: ASD 14±4 mm Hg, controls 10±5 mm Hg, P=0.01; peak: ASD 25±9 mm Hg, controls 14±9 mm Hg, P=0.0001). The right atrial v wave pressures were increased at rest but not at peak exercise. The transmural filling pressure gradient (TMFP) was higher at peak exercise among patients with ASD (10±6 mm Hg, controls 7±3 mm Hg, P=0.03). One third of patients with ASD demonstrated an abnormal hemodynamic exercise response defined as mean pulmonary artery wedge pressure ≥25 mm Hg and/or mean pulmonary artery pressure ≥35 mm Hg at peak exercise. These patients had significantly elevated peak right and left atrial a wave pressures, right atrial v wave pressures, pulmonary artery wedge v wave pressures, and transmural filling pressure compared with both controls and patients with ASD with a normal exercise response. Conclusions Patients with corrected ASD present with elevated right and in particular left atrial pressures at rest and during exercise despite preserved peak exercise capacity. Abnormal atrial compliance and systolic atrial function could predispose to the increased long-term risk of atrial fibrillation. Registration Information clinicaltrials.gov. Identifier: NCT03565471.

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Right-side pulmonary agenesis with atrial septal defect in adult.

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