Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease

Saalouke, Michael G.; Shapiro, Stephen R.; Perry, Lowell W.; Scott, Lewis P.

doi:10.1016/s0002-9149(77)80102-1

Cited by 50 publications

(22 citation statements)

References 5 publications

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“…Babb et al 8 . and Saalouke et al 26 . documented elevated PAP on cardiac catheterization in these patients.…”

Section: Discussionmentioning

confidence: 84%

“…documented elevated PAP on cardiac catheterization in these patients. Saalouke et al 26 . suspected PAPVC on chest X‐ray and confirmed the diagnosis with cardiac catheterization and oximetry studies.…”

Section: Discussionmentioning

confidence: 94%

“…Congenital heart disease associated pulmonary hypertension is included in group I of the 4th World Symposium on Pulmonary Hypertension 11 . Isolated anomalous PV return has been associated with PAH 8,26 . Babb et al 8 .…”

Section: Discussionmentioning

confidence: 99%

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Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Sahay

Krasuski²,

Tonelli

2012

Respirology

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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 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 the PAPVC. Group I included patients with PAPVC (with or without 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 mmHg. Group I had a mean PAP of 23.6 ± 6.6 mmHg as compared to 33.7 ± 16.5 mmHg 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 hemodynamic findings. Conclusions Patients with PAPVC (with or without PFO) in the absence of other CHD had normal PAP unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.

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“…Babb et al 8 . and Saalouke et al 26 . documented elevated PAP on cardiac catheterization in these patients.…”

Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 94%

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Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Sahay

Krasuski²,

Tonelli

2012

Respirology

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“…None of our patients developed pulmonary vascular obstructive disease (PVOD). However, there have been some patients with isolated PAPVC who have developed PVOD after a long time [7,20]. The mechanisms responsible for the development of PVOD are not clear, but PVOD may develop because of increased abnormal flow.…”

Section: Resultsmentioning

confidence: 99%

“…The mechanisms responsible for the development of PVOD are not clear, but PVOD may develop because of increased abnormal flow. In PAPVC, the pulmonary flow does not always distribute equally to each pulmonary segment because of the slight differences in atrial pressures and ventricular distensibilities which may cause a greater decrease in resistance in the anomalously draining lung tissue than in the area draining normally [20]. Follow-up plans should include care of progressive PVOD; thus, serial cardiac evaluations may be necessary even for patients with small left-to-right shunts.…”

Section: Resultsmentioning

confidence: 99%

Diagnosis and Management of Partial Anomalous Pulmonary Venous Connection

1998

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Medical records, angiograms and operative records of 28 patients with partial anomalous pulmonary venous connection (PAPVC) were reviewed. Twenty patients had one anomalous pulmonary vein (APV), and 8 had more than two APVs. Twenty-five patients (89%) had APVs originating from the right lung, 2 (7%) from the left lung and 1 (4%) from both lungs. In the 25 patients with APVs originating from the right lung, 9 had APVs draining into the superior vena cava (SVC), 13 into the right atrium (RA), 1 into the inferior vena cava (IVC) and 2 into both the SVC and RA. In the 2 patients with APVs originating from the left lung, 1 had APVs draining into the RA, and the other had APVs draining into the innominate vein. The patient with APVs originating from both lungs had connection to the IVC. Twenty-three patients (82%) had additional cardiovascular defects. Surgery was performed in 13 patients who had pulmonary/systemic flow ratios greater than 2.0. The patients have done well after surgery. In 7 patients, we were unable to accurately determine the number or sites of drainage of APVs prior to surgery. We conclude that selective pulmonary angiography is indispensable for the accurate diagnosis of PAPVC.

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