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EP.292
Partial Anomalous Pulmonary venous Return ( PAPVR) Case Report

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Summary

A 61 year-old male underwent cannulation of the left jugular vein following admission to Intensive care for severe shortness of breath. A post procedure check chest XR(CXR) showed the central venous catheter(CVC) tracing a path along the left heart border. Review of CT Pulmonary Angiogram (CTPA), performed prior to admission to the ICU and the CVC insertion, demonstrated ananomalous left upper lobe pulmonary vein draining into the left subclavian vein, with no evidence of pulmonary systemic shunt. Partial Anomalous Pulmonary venous connection is a rare, however, failure to recognise this condition could lead to confusing and misleading information when using a CVC to guide treatment.


Introduction

Partial anomalous pulmonary venous return (PAPVR) is a vascular anomaly, where one or more pulmonary vein drains into right sided circulation instead of the left atrium. It is a rarely seen anomaly in adults. We report a case of PAPVR which was found incidentally on insertion of a left sided internal jugular CVC.


Case Report

A 61 year old male, presented with a one month history of severe shortness of breath and chest pain. He had no history of cough, fever or recent travel abroad.He had a background of rheumatoid arthritis for 10 years and an ST elevation myocardial infarction with primary percutaneous intervention 4 years prior to admission. He was on  Methotrexate for 10 years, stopped 2 months prior to admission due to a chest infection. On Examination, he was hypoxic with oxygen saturation of 90% on high flow oxygen therapy, the rest of his vital signs were stable. Clinically the patient had crepitations bilaterally with no evidence of volume overload. A CTPA ruled out pulmonary embolism, but showed ground glass changes throughout both lungs.Pneumocystis Carinii was grown from sputum culture and he was started on Co-trimoxazole . His respiratory function continued to deteriorate and he was transferred to the ICU for supportive treatment. Central venous cannulation of the right internal jugular vein under ultrasound guidance was complicated by an accidental arterial puncture. Subsequent Ultrasound-guided cannulation of the left internal jugular vein was uneventful, and the lumen of the left internal Jugular vein was noticeably more distended and easier to locate than the right.The post procedure chest x-ray showed the central venous catheter(CVC) tracing a path along the left heart border. With the patient on 2litres of oxygen via nasal cannulae, a blood sample taken from the CVC showed a pO2 of 37.2kPa and pCO2 of 4.5kPa. The peripheral arterial blood sample  taken simultaneously, showed a pO2 of 9.07kPa and pCO2 of 4.6kPa. The transduced waveform from the central line had the appearance of pulmonary artery trace. The CT pulmonary angiogram (CTPA) which was performed to exclude pulmonary embolism a few days prior to central venous cannulation was reviewed by the radiologist at the request of the ICU team. Closer inspection of the CTPA revealed an anomalous left upper lobe pulmonary vein draining into the left subclavian vein. Although portion of the subclavian vein run very close to a pulmonary arterial branch, there was no evidence of arterio-venous malformation( AVM) and the contrast density in the pulmonary artery and the pulmonary vein was different.

 

Discussion

Partial anomalous pulmonary venous return (PAPVR) occurs when some of the pulmonary veins connect to the right atrium or one of its venous tributaries rather than the left atrium with a reported incidence of 0.4-0.7%{[1]}.Most of the anomalous pulmonary veins arise from the right lung and drain into the superior vena cava (SVC).  A small minority of PAPVR approximately 10% are left sided.Only 3% of left sided PAPVR cases have been reported to drain from the left superior pulmonary vein into the left Innominate vein There is no definitive data to confirm incidence amongst males compared to females and there is no evidence of genetic predisposition or teratogens in the genesis of PAPVR{[2]}.It can be associated with cardiac malformations including atrial septal defect ( ASD), with no more than 3% of the cases presenting with intact intra atrial septum{1}. The number of the pulmonary veins draining into the systemic circulation and the magnitude of the left to right shunt are usually the main determinants of the clinical outcome. An isolated PAPVR is usually small, without haemodynamic compromise and rarely requires surgical correction as it is largely asymptomatic.

Pulmonary hypertension, pulmonary vascular disease, and right sided heart failure may occur secondary to a longstanding left to right shunt and in such patients surgical correction might be necessary. It’s more commonly an incidental finding in asymptomatic patients undergoing pulmonary vascular studies for other indications.

In our case the, the left internal jugular vein was more filled and easier to cannulate compared to the right. The oxygen saturation from CVC blood sample was higher than that of the arterial line and the wave form similar to that of pulmonary circulation. Such findings together with the radiological evidence  of abnormal CVC placement,  caused a certain amount of confusion as to the actual location of the CVC. We hope that this report will highlight PAPVR as a possible cause of CVC “displacement” and anomalous venous blood gas analysis results.

 




 References

 {[1]}.Fragata J et al. Partial Anomalous Pulmonary Venous Connection: Surgical Management. World Journal for Paediatric and Congenital Heart Surgery 2013 4: 44

 {[2]}.Nath R, Murphy W, Aronson B et al. Rare case of left upper lobe partial anomalous pulmonary venous connection. Radiology Case. 2013 June;7(6):9-14

 

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