PURPOSE: Isolated partial anomalous pulmonary venous connections (PAPVC) have been implicated to cause the pulmonary hypertension (PH), however this condition is often overlooked in the diagnostic work up of PH patients. We sought to identify the prevalence of pulmonary hypertension in the patients with isolated PAPVC.
METHODS: We retrospectively analyzed data from the Adult Congenital Heart Disease database at the Cleveland Clinic, Cleveland, Ohio from the October 2005 to October 2010. We included all patients with the diagnosis of PAPVC with or without other congenital heart defects. We excluded all patients with previous corrective surgeries.
RESULTS: We identified 14 (1.7%) patients with PAPVC and no previous corrective cardiac surgeries. These 14 patients were divided into two groups. Group 1 included patients with isolated PAPVC (three of them also had PFO). Group 2 included patients with PAPVC associated with other congenital heart defects. The mean PAP in all patients (n=14) was 29.5 ± 13.8 mmHg. Group 1 had a mean PAP of 23.6 ± 6.6 mmHg as compared to 33.7 ± 16.5 mmHg for group 2 (p = 0.34). The two patients that had PH in group 1 had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the hemodynamic findings.
CONCLUSIONS: Most of the patients with isolated PAPVC in our study had normal pulmonary artery pressure unless they have two pulmonary veins with anomalous return or associated conditions known to cause PH.
CLINICAL IMPLICATIONS: Early identification of PAPVC can prevent the later development of PAH in some patients. Patient screening is recommended on a large scale.
DISCLOSURE: The following authors have nothing to disclose: Sandeep Sahay, Adriano Tonelli, Richard Krasuski
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