Keywords: Fontan operation
Background
Tricuspid atresia accounts for about 0.7% of all congenital heart diseases. The tricuspid valve is absent, and there is no anatomic connection and direct communication between the right
atrium and the right ventricle.
Case description
This 27-year-old female after Fontan operation is presented. The patient was born at term but was cyanotic. Diagnosis was made of tricuspid atresia with atrial septal defect (ASD) and ventricular septal defect (VSD). At age of 3.5 years a Fontan circulation was established by means of an atriopulmonary anastomosis (direct anastomosis between the right atrium and the confluent pulmonary artery (PA) using vascular homograft. She was seen for routine follow-ups during childhood. At age of 24 years a clinical follow-up was performed (without catheterization) – at that time she was classified in NYHA class II, arterial oxygen saturation was 93%.
Since that time she had been in good condition, could walk about 100 m without stopping but she could not run very far. Deteriorated physical capacity was noticed 1.5 year ago but she was still stable. She has been experiencing palpitations for the last 1.5 years – atrial flutter, and paroxysmal atrial tachycardia requiring intravenous antiarrhythmic medication. The subclinical hypothyreosis, hypogonadism with normal level of gonadotropin, asthma and epilepsy were also diagnosed. Current medication she receives is: Bisoprolol 2.5mg daily, Warfarin-under INR control, Euthyrox 25ug daily, Hydrocortison 10mg daily, Vigantoletten (Colecalciferolum) 1.0 g daily, Depakine chrono 500-300-500mg, Alvesco (cyclezonid) 2 puffs daily.
The ECG revealed sinus rhythm 60 bpm with supraventricular ectopic beats, T-wave inversions in I, avL, V2-V5 and first degree AV block. First Holter monitoring (04.2011) showed 12000 supraventricular ectopic beats but another one (07.2011), after changing a beta-blocker showed only 356 supraventricular ectopic beats, 1 SVT. Biochemical analysis showed high level of NT-proBNP 695 pg/ml (<125pg/ml). In 6MWT she walked 495m without desaturation. She underwent cardiopulmonary testing- maximum oxygen uptake at peak exercise (O2peak) was low (15.15ml/kg/min) and carbon dioxide production (VE/VCO2 slope) was 39.71 which is known to be a bad prognostic factor for cardiac hospitalization and mortality.
TEE showed severely enlarged right atrium with spontaneous echo contrast. The interatrial septum with Gore-Tex patch was without leak and the connection between the RA and PA was widely patent.
We performed CT angiography which confirmed the presence of severely enlarged right atrium measuring 70×75x82 mm connected with PA on the right-posterior site. No thrombus was present in the RA. There was a small flow between the hypoplastic RV cavity and PA.
We decided to perform catheterization to visualize cardiac structures and obtain pressure measurements. Contrast injected to the right atrium showed enlarged right atrium with contrast medium flowing to pulmonary arteries, no stenosis was present. The pressures in vena cava superior and inferior, right atrium and pulmonary arteries were high: 21mmHg, cardiac output (Fick method) was 4.67 l/min and cardiac index 2.54 l/min/m2. Pulmonary vascular resistance was 1.8 Wood units.
The patient was selectd for surgical treatment: extracardiac TCPC (total cavopulmonary connection) conversion due to enlarged right atrium with onset of atrial arrhythmia.
References
1. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010 Dec;31(23):2915-57.
Expert’s conclusions:
Conversion to TCPC (total cavopulmonary connection) should be considered.
Authors:
Mroczek E. MD, PhD1, Podolec J. MD3, Kusa J. MD, PhD2
Experts:
Pająk J.MD, PhD4, Prof. Skalski J.MD, PhD5, Tomkiewicz-Pająk L.MD,PhD6, Kordon Z.MD, PhD7, Kopeć G.MD, PhD6, Prof. Podolec P. MD, PhD6
1 Cardiology Department, County Hospital Wrocław
2 Pediatrics Cardiology Department, County Hospital Wrocław
3 The Cardiovascular Intervention Center, John Paul II Hospital, Krakow, Poland
4 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
5 Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Jagiellonian University,
Krakow, Poland
6 Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II
Hospital, Krakow, Poland
7 Department of Pediatric Cardiology, Polish–American Children’s Hospital, Krakow, Poland
