Background
Nowadays we can observe increasing number of grown-up congenital heart diseases, due to remarkable improvement in survival of pediatrics patients. Most of adult patients have undergone palliative operation in first years of life. Patients with univentricular heart present a multiple variety of malformations: missing or hypoplastic left or right ventricle, connected with corresponding valvular and great vessels anomalies. In such cases, most patients have restricted pulmonary blood flow, palliatively treated in childhood by surgically made systemic-to-pulmonary shunts. Examples of palliative systemic-pulmonary shunts are: most commonly Blalock-Taussig shunt (subclavian to pulmonary artery) and rarely: modified Blalock-Taussig shunt (vascular prosthesis), Waterston shunt (ascending aorta – to – right pulmonary artery anastomosis), Potts shunt (descending aorta – to – right pulmonary artery anastomosis) and central shunt. Echocardiography seems to be the most adequate, first-line diagnostic tool, as for a diagnostic, as well as for a follow-up approach.
Case presentation
We present a case of 58-year old male with congenital cyanotic heart disease (pulmonary arterial hypertension suspected), with diagnosed double inlet left ventricle (DILV), transposition of the great arteries (d-TGA), ventricular septal defect (VSD) and subvalvular pulmonary artery stenosis (PS). In childhood he have undergone palliative treatment – systemic-to-pulmonary shunts: Blalock-Taussing at the age 12 and Waterston at the age of 31. At admission current states were typical: central cyanosis, dyspnoe, fatigue: HF NYHA III / WHO III (progression NYHA I/II à III in the last 2 years), polycythemia, thrombocytopenia. He suffers also from DM type 2, arterial hypertension and hyperuricemia. In physical examination patient’s height was 182 cm, weight – 75 kg and BMI – 22,6 kg/m². He had significant central cyanosis, clubbing of the nail beds and in the auscultation: loud P2, systolic murmur over the III left intercostal space radiating upward and to the right and over the apex (due to AV regurgitation). His blood pressure was 135/90 mmHg and HR nearly 80 bpm. ECG tracing showed right axis deviation, peaked P waves, PAT 2:1, QRS rate 82 bpm and QRS duration 160ms, while the Holter monitoring – one episode of nsVT. Blood biochemical analysis revealed markedly elevated NT-proBNP level – 8722 pg/ml [N: 0 – 125], GGTP – 206 U/l [N < 60] and blood morphology deviations: poorly elevated erythrocytes – 5,55×10^6/ul [N: 4,50 – 5,50], hemoglobin – 18,12 g/dl [N: 13,00 – 18,00], hematocrit – 54,20% [N: 40,00 – 54,00], and decreased platelet count – 107×10^3/ul [N: 135 – 350]. We performed 6-minute walking test. The initial desaturation was escalated after 6 minutes of walking (68%à60%), while BP and HR – increases after exertion (HR 82 bpm à 113 bpm; BP 140/80 mmHg à 160/95 mmHg). He cover the distance of 390 m. In ergospirometry his VO2max was 14 ml/kg/min. The echocardiographic examination showed common ventricle with residual intraventricular septum diameter of 17mm, EDD – 80mm, ESD – 69mm, PW – 11mm, RA area 32cm², LA area – 28cm². Tricuspid, mitral and pulmonic valves, as well as aortic orifice from residual RV, moderate regurgitations were visualized. The diameter of right pulmonary artery was 24mm, of left pulmonary artery – 35mm and of common pulmonary trunk diameter – 28mm. Left ventricle (LV) systolic function was slightly decreased with global ejection fraction (EF) of 45%. Multislice computed tomography revealed the diameter of the Waterstone shunt – 8.9mm, dilatation of right and left pulmonary arteries and narrowing of the left pulmonary artery. There was shunt kinking with probably preferential flow distribution of most shunt to the right lung, thus PAH was suspected. We excluded other typical Waterstone shunt complications, such as: narrowing of the right PA at the anastomosis site, acquired hypoplasia of the left PA, shunt obstruction. Blalock-Tausing shunt was not visualized. Coronary angiography was normal. All performed investigations confirmed anatomy and diagnosis of congenital heart disease, leading to PAH syndrome occurrence. He was treated by walsartan 1x160mg, metoprolol ZK 1×47.5mg in the morning and 1/2×47,5mg in the evening, digoxin 1×0.25mg, torasemide 1x10mg, spironolacton 1x50mg, gliclazyde 1x30mg, allopurinol 1x100mg and acenocumarol. We decide to add endothelin receptor antagonist 2×62.5mg, with good drug tolerance and response – NYHA reduction from class III to II in one month, decrease of NT-proBNP level (1232 vs 8722pg/ml).
Current guideline
There are limited suggested diagnostic and therapeutic algorithms. The key diagnostic tool is echocardiography, while medical treatment should be individualized (i.e. PAH treatment), dependent on current symptoms, pharmacotherapy response and previous case history. In all cases, life-long follow-up in specialized centers is necessary.
References
1. Baumgartner H, Bonhoeffer P, De Groot NM et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010;31:2915-57.
2. Levin DC, Fellows KE, Sos TA. Angiographic demonstration of Complications Resutling from the Waterstone procedurę. Am J Roentgenol 1978; 131: 431-37.
3. BeBoer A. The Waterstone shunt: a commentary. Ann Thorac Surg 1987; 44: 326-7.
Expert’s comments:(Written authorization required from each expert)
1. J. Podolec
Waterstone operation is a very useful but old procedure. With time, this kind of shunts tend to develop complications. In this case pulmonary hypertension might be caused by changes in Waterstone shunt. Knowledge about real pulmonary artery pressure is desirable. Therefore, right heart catheterization performed by an experienced team is now important.
2. Kopeć
Clinical worsening of this patient for the last 2 years may be related to development of pulmonary hypertension. Additionally, kinking of the Waterstone shunt and blood flow redistribution may an add to observed deterioration. Right heart catheterization would be very useful at this stage. Reaction on bosentan is surprisingly good. Therefore, continuation of this treatment should be recommended.
Expert’s conclusions:
Therapy with bosentan will be continued. Right heart catheterization, performed by an experienced team will be scheduled.
Authors:
Katarzyna Mizia-Stec MD, PhD1, Joanna Wieczorek, MD1
Experts:
J. Podolec, Kopeć
1 I Department of Cardiology, Medical University of Silesia, Katowice, Poland
