Background
Tetralogy of Fallot is a one of congenital heart defects involving four anatomical abnormalities: pulmonary stenosis or pulmonary atresia, overriding aorta, ventricular septal defect, right ventricular hypertrophy. It is the most common cyanotic heart defect. The main symptoms are a bluish coloration of the skin caused by blood low in oxygen (cyanosis), a heart murmur and clabbing of fingers. Tetralogy of Fallot with atresia of the left pulmonary artery is presented by 10% of patients.
Case presentation
We present a case of a 33-years old caucasian woman, 19 years after surgical correction of pulmonary atresia with ventricular septal defect. She was diagnosed with pulmonary artery atresia and VSD in the age of 13 years old. The surgical correction of congenital heart defect was performed one year later, in 1993. The aorta ascending was connected with the right pulmonary artery with the dacron conduit and the right ventricle with the left pulmonary artery by the dacron conduit. For the next 18 years she was asymptomatic. During this time she was pregnant twice, and had two uneventful cesarean sections. She has two children, an autistic boy and a girl with suspicion of patent ductus arteriosus. The patient was hospitalized due to heart defect in Warsaw in 2009. She was diagnosed with a stenosis of conduit connected the right ventricle with the left pulmonary artery and with an occlusion of the second conduit connected the ascending aorta with the right pulmonary artery. She was qualified to the conservative treatment. However, she started complaining about increasing fatigue and dyspnoe on exertion for the last year. At presentation her vital signs were as follows: heart rate of 100 bpm, arterial blood pressure of 90/50 mmHg; oxygen saturation was only 68% (on air). On physical examination we found typical signs of prolonged hypoperfusion such as peripheral cyanosis, systolic murmur of 5/6 in Levine’s scale, mild liver enlargement and clubbed of fingers. On the ECG she was in sinus rhythm with 1-3 mm ST depression in leads I, II, III as well as an elevated J point in V1-V5. The echocardiographic examination revealed enlargement of the right ventricle and the right atrium, persistent VSD with 24 mm in diameter, the atresia of massively calcified pulmonary valve without any flow, and misplaced aorta, which is supplied from the left and the right ventricle. We did not find anything remarkable on the 24-hour Holter study. In the laboratory tests we observed typical signs of the poliglobulia such as significantly elevated levels of red blood cells (7.6 mln per ), elevated hematocrit ( 65,8 %) and hemoglobin (17,6 g/dl). To objectively assess her exercise capacity we performed 6-minute walk test, which revealed oxygen desaturation to 44%. On cardiopulmonary exercise test her peak oxygen uptake was only 9.5 ml/kg/min and metabolic equivalent for carbon dioxide of 58. The Angio-CT confirmed massive pulmonary valve calcification without flow, patent but critically tapered conduit (minimal lumen diameter 6 mm per 6 mm) from the right ventricle to the left pulmonary artery, and non-functioning conduit between ascending aortic and right pulmonary artery. Which option is the best for this patient: a re-operation, a conduit PCI or a medical treatment?
Current guidelines
ESC Guidelines for the diagnosis and treatment of Heart Failure from 2010 not define clearly of procedure with patient after surgical correction of ToF with a stenoctic or occlusive conduit.
References
1. Kenneth Dickstein, Panos E. Vardas, Angelo Auricchio et al. 2010 Focused Update of ESC Guidelines on device therapy in heart failure. European Heart Journal 31:2677–2687
Expert’s comments:(Written authorization required from each expert)
1. Bogusław Kapelak, MD, PhD
The re-operation of heart failure is doable using a biological conduit, without VSD closure.
2. Jacek Kołcz, MD, PhD
The helix conduit with a valve is recommended in this case.
3. Jacek Pająk, MD, PhD
The stenotic conduit can be damaged by any manipulation.
Expert’s conclusions:
Treatment of this patient should be a surgery with an implantation of a biological conduit with a valve between the right ventricular and the pulmonary artery of a diameter of the left pulmonary artery. It’s a high-risk surgery.
Authors:
Dawid Kudliński MD1, Magdalena Kostkiewicz MD, PhD2, Agata Leśniak-Sobelga MD1, Lidia Tomkiewicz-Pająk MD, PhD1, Tomasz Miszalski-Jamka MD, PhD2, Prof. Piotr Podolec MD, PhD1.
Experts:
Bogusław Kapelak MD, PhD3, Jacek Kołcz MD, PhD4, Jacek Pająk MD, PhD5
1Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
2One-Day Diagnostic Department, John Paul II Hospital, Krakow
3Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
4Department Cardiac Surgery Polish – American Children’s Hospital, Krakow, Poland
5Independent 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
