Background
Pulmonary atresia with ventricular septal defect (PA + VSD) is a cyanotic congenital heart disease, also called Tetralogy of Fallot with pulmonary atresia. The intracardiac anatomy is similar to tetralogy but there is no direct communication between the right ventricle and pulmonary arteries. Major problems with surgical treatment are related to complexity of the pulmonary vascular bed that may make repair impossible.
Case presentation
We present a case of a 50-year old woman with congenital heart disease who was admitted to our Centre in July 2012 for cardiologic evaluation. The patient underwent noninvasive diagnostic and first catherisation at the age of 28 (aorta: preassure 160/84, mean 112mmHg, oxigen saturation 84%; left ventricle: 160/8, mean 16mmHg, 87%; right ventricle: 120/8, mean 12mmHg, 64%; right atrium: a wave 8mmHg, v wave 6mmH, 56%). Then congenital heart disease was diagnosed as pulmonary valvue atresia with ventricular septal defect with right-to-left shunt and common atrerial trunk. The conservative treatment was instituted. On admission to our Centre the patient complained of easy fatigue and low exercise tolerance (NYHA III class). Physical examination revealed central cyanosis, clubbed fingers, systolic murmur 3/6 in Levine and basal crepitation on the left .Laboratory workup showed elevated levels of hemoglobin, hsCRP, troponin T and NT-proBNP. The distance in 6-minutes walking test was 522m with reducing pulse oximetry from 85 to 77% and dyspnoea estimated at 7 in Borg scale. Echocardiography showed pulmonary valvue atresia, ventricular septal defect, overriding aorta with biventricular connection and dilatated ascending aorta to 52mm. CT angiography of thoracic aorta revealed complicated anatomy of vessels in thoracic cage, especially narrow and hypoplastic major aortopulmonary collateral arteries (MAPCAs) that arise from descending part of aorta and left subclavian artery.
Current guidelines
Patients with PA +VSD surviving unrepaired to adulthood may have been considered unoperable years ago, but may actually benefit from modern surgical or interventional procedures. Therefore they should be critically re-evaluated in specialized centres. However, many unrepaired patients may not be suitable for further surgery. Patients with segmental PAH have been considered for targeted. PAH therapy, although there is a lack of data [1].
References
1.ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957
Expert’s comments:(Written authorization required from each expert)
1. Bogusław Kapelak, MD, PhD
There is a shortage of experience in adult clinic. Therefore, detailed evaluation is required together with multidisciplinary consideration.
2. Jacek Pająk, MD, PhD
Pulmonary vessles are narrowed, pulmonary hypertension is possible, the surgical procedure is impossible to perform.
3. Prof. Lesław Szydłowski, MD, PhD
The patient is not for surgical procedure, the congenital heart disease should be classified as Tetralogy of Fallot with pulmonary atresia, evaluation of preassure in MAPCA’s is important.
4. Grzegorz Kopeć, MD, PhD
There are no indications to institute specific treatment of PAH.
5. Danuta Sorysz, MD, PhD
In case there is need for palliative dilatation procedure of MAPCAs should be considered.
Expert’s conclusions:
Further conservative treatment has been recommended. There is no possibility of confirming pulmonary hypertension and therefore there are no indications to repeat catherisation and institute specific treatment of pulmonary hypertension.
Authors:
Agnieszka Sarnecka MD1, Grzegorz Kopeć MD. PhD1, Prof. Piotr Podolec MD. PhD1
Experts:
Bogusław Kapelak MD, PhD2, Jacek Pająk MD, PhD3, Prof. Lesław Szydłowski MD, PhD4, Grzegorz Kopeć MD, PhD5, Danuta Sorysz MD, PhD6.
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
3Independent Public Clinical Hospital No.6 Medical University of Silesia in Katowice, Upper Silesia Center of the children’s Health. John Paul II, Pediatric Heart Surgery Department Poland
4Department of Pediatric Cardiac Surgeryy, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
5Department of Heart and Vascular Disease, John Paul II Hospital, Kraków, Poland
6II Departement of Cardiology University Hospital in Cracow
