Partial anomalous pulmonary venous connection and pulmonary arterial hypertension
- 18 April 2012
- journal article
- research article
- Published by Wiley in Respirology
- Vol. 17 (6), 957-963
- https://doi.org/10.1111/j.1440-1843.2012.02180.x
Abstract
Background and objective: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. Methods: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, USA between October 2005–2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. Results: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. Group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. Conclusions: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.Keywords
This publication has 24 references indexed in Scilit:
- Isolated Partial Anomalous Pulmonary Venous Connections in Adults: Twenty-Year ExperienceCongenital Heart Disease, 2010
- Magnetic resonance imaging is the diagnostic tool of choice in the preoperative evaluation of patients with partial anomalous pulmonary venous returnThe International Journal of Cardiovascular Imaging, 2006
- Look before you close: Atrial septal defect with undiagnosed partial anomalous pulmonary venous returnCatheterization and Cardiovascular Interventions, 2005
- Necessity for Evaluation of Anomalous Pulmonary Venous Return Before Percutaneous Closure of Atrial Septal DefectsAngiology, 2005
- Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return evaluated by multislice CTHeart, 2004
- Isolated partial anomalous pulmonary venous connection: diagnostic value of suprasternal color flow imaging and contrast echocardiographyJournal of the American Society of Echocardiography, 2003
- Incidence and Size of Patent Foramen Ovale During the First 10 Decades of Life: An Autopsy Study of 965 Normal HeartsMayo Clinic Proceedings, 1984
- Isolated Partial Anomalous Venous Connection: A Congenital Defect with Late and Serious ComplicationsThe Annals of Thoracic Surgery, 1981
- Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease.Circulation, 1978
- Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive diseaseThe American Journal of Cardiology, 1977