(IV-2A.1) (English) 18-year-old patient with tetralogy of Fallot and coronary artery abnormality Smaś-Suska M.MD, Tomkiewicz-Pająk L. MD,PhD, Tyrka A. MD, Olszowska M. MD, PhD, Miszalski-Jamka T. MD, PhD, Podolec J. MD

Keywords: coronary artery abnormality, tetralogy of Fallot

Background
Tetralogy of Fallot is one of the most common cyanotic congenital cardiac diseases. This heart defect consists of ventricular septal defect, obstruction of the right ventricular outflow tract, overriding aorta and right ventricular hypertrophy. About 3% of patients have anomalous left anterior descending coronary artery which may necessitate a conduit type of repair. Conduits connect right ventricle and main pulmonary artery. There are two types of conduits: valved and non-valved. About 40% of patients with conduits do not require reoperation within 20 years whereas the remaining patients need a surgical procedure due to conduit failure.

Case description
This 18-year-old patient with tetralogy of Fallot and coronary artery abnormality was admitted to the hospital. At 4 years of age total corrective surgery was performed which included ventricular septal defect closure and implantation of a conduit between right ventricle and main pulmonary artery. A year later angioplasty of pulmonary artery was performed due to aneurysm. On admission the patient reported chronic fatigue, reduced exercise tolerance, and he was in NYHA class II/III. On examination systolic murmur in second intercostal space was heard. ECG revealed sinus rhythm 70 bpm, right axis deviation, Q wave in III, aVF, inverted T waves in I, aVL and signs of RV enlargement. The cardiopulmonary exercise test was discontinued after 3 min 20 s due to fatigue and dyspnea and showed reduced exercise tolerance with maximal oxygen consumption VO2 max 12.4 ml/kg/min. The echocardiogram showed normal size and function of the left ventricle with residual ventricular septal defect, right ventricular and atrial enlargement, hypoplastic pulmonic valve with calcification, mild tricuspid and pulmonary regurgitation, moderate aortic regurgitation. Maximal pressure gradient measured in the right ventricular outflow tract was 55/32 mmHg. Furthermore, the conduit between right ventricle and main pulmonary artery with turbulent flow was visualized. The CT heart scan showed subvalvular stenosis (1,7×0,6 cm), hypoplastic pulmonic valve (2,0×0,7cm), conduit connecting right ventricle and main pulmonary artery with calcification in the middle part of the conduit reducing its lumen to 1.9 x 1.5 cm. The examination confirmed anomalous coronary artery.

Guidelines
2010 ESC Guidelines for the management of grown-up congenital heart disease:
→ Indication for intervention in patients after repair of tetralogy of Fallot.
1. Reoperations should be performed in symptomatic patients with severe pulmonary regurgitation and/or stenosis (RV systolic pressure >60 mmHg, TR velocity >3.5m/s)
Class I C.
2. Reoperations should be considered in asymptomatic patients with severe pulmonary regurgitation and/or pulmonary stenosis when at least one of the following criteria is present:
– Decrease in objective exercise capacity
– Progressive right ventricular dilation
– Progressive right ventricular systolic dysfunction
– Progressive tricuspid regurgitation (at least moderate)
– RVOTO with RV systolic pressure >80 mmHg (TR velocity >4.3 m/s)
– Sustained atrial/ventricular arrhythmias
Class IIa C.
→ Indication for intervention in patients with right ventricular to pulmonary artery conduits:
1. Symptomatic patients with RV systolic pressure >60 mm Hg ( TR velocity >3,5 m/s; may be lower in case of reduce flow) and/or moderate/ severe PR should undergo surgery.
Class I C.
2. Asymptomatic patients with severe RVOTO and/or severe PR should be considered for surgery when at least one of the following criteria is present:
– Decrease in exercise capacity (CPET)
– Progressive RV dilatation
– Progressive RV systolic dysfunction
– Progressive TR ( at least moderate)
– RV systolic pressure > 80 mm Hg (TR velocity >4,3 m/s)
– Sustained atrial/ventricular arrhythmias
Class IIa C

References
1. ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957
2. Mohammadi S, Belli E, Martinovic I, Houyel L, Capderou A, Petit J, Planche C, Serraf A. Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation. Eur J Cardiothoracic Surg 2005; 28:217-222

Experts’ conclusions
The patient was selected for observation and catheterization.

Authors:
Smaś-Suska M.MD1, Tomkiewicz-Pająk L. MD,PhD1, Tyrka A. MD1, Olszowska M. MD, PhD1, Miszalski-Jamka T. MD, PhD2, Podolec J. MD3

Experts:
Prof. Skalski J. MD, PhD4, Pająk J. MD, PhD5, Kordon Z. MD, PhD6, Prof. Biederman A. Md, PhD7, Prof. Sadowski J. MD, PhD8, Kopeć G. MD, PhD1, Staszecka-Prokop A. MD, PhD9, Bederski K. MD, Prof. Podolec P. MD, PhD1.

1 Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
2 Center for Diagnosis, Prevention and Telemedicine, John Paul II Hospital, Krakow, Poland
3 The Cardiovascular Intervention Center, John Paul II Hospital, Krakow, Poland
4 Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
5 Independent 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
6 Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
7 Department of Cardiac Surgery, Institute of Cardiology, Warsaw, Poland
8 Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
9 Department of Pulmonology in John Paul II Hospital, Krakow
10 Department of Thoracic Surgery in John Paul II Hospital, Krakow

 

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