Keywords: correction of Tetralogy, pulmonary valve regurgitation, left pulmonary branch stenosis
Background
Tetralogy of Fallot (ToF) is a cyanotic congenital heart disease. Almost all unoperated patients used to die before 40 years of age. Prognosis after surgical correction is good, with
a 35-year survival of 85%. Surgery is carried out to close ventricular septal defect and relieve right ventricular outflow tract obstruction. Patients who have reached adulthood after earlier repair of ToF frequently develop complications such as severe pulmonary regurgitation, right ventricular outflow tract obstruction, right ventricular dilation and dysfunction, residual ventricular septal defect, left ventricular dysfunction, aortic root dilation with aortic regurgitation, endocarditis, atrial and ventricular tachycardia, sudden cardiac death.
We present a patient after ToF repair with complex complications.
Case presentation
This 31-year-old man with Tetralogy of Fallot underwent surgical correction with transannular pulmonary patch at 3 years of age. The patient had not been systematically monitored by the cardiologist. Since 2009 the patient has been manifesting the symptoms of heart failure. In September 2011 cardiac catheterization was performed with the following results: right atrium 11/17/11 mmHg, right ventricle 64/8/18 mmHg, pulmonary artery 44/14/24 mmHg, left ventricle 102/-5/17 mmHg, aorta, PCWP 16/17/15 mmHg; ventricular septal defect and severe stenosis of left pulmonary artery branch; Sat O2: superior vena cava 36.7 %, inferior vena cava 52.8 %, right atrium 50.8 %, right ventricle 56%, pulmonary artery 59.8%, left ventricle 92%, aorta 93%. There were multiple communications in the lungs and liver facilitating a right-to-left shunt that caused cyanosis. On admission the patient’s general condition was poor. Physical examination revealed slight enlargement of the liver and spleen, odema, and ascitis.
Laboratory studies:
E – 5730000, L- 5120, Hg- 15.6 g/dl, Ht- 47%, PLT- 170 000, INR- 0,89, Alb- 24.6 g/l, ASPAT- 69 U/L, ALAT- 69 U/L, Protein – 46.7 g/L, ALP – 343, pBNP – 1106 pg/ml
Abdominal ultrasound showed abnormal liver with irregular contours, varying echogenicity and reduced vascularity. Holter monitoring revealed atrial fibrillation. Echocardiography showed impaired function of the right ventricle, major pulmonary valve regurgitation, major tricuspid valve regurgitation and ventricular septal defect with left-to-right shunt. MR showed impaired function of the right ventricle and severe stenosis of the left pulmonary branch. High protein diet, diuretics, albumin were ordered. After a few days clinical improvement was observed.
Current guidelines
According to ESC Guidelines the indications for intervention after repair of Fallot’s syndrome include:
1. Aortic valve replacement in patients with severe aortic regurgitation with symptoms or signs of left ventricular dysfunction (I C)
2. Pulmonary valve replacement in symptomatic patients with severe pulmonary regurgitation and/or stenosis (right ventricular systolic pressure >60 mmHg, tricuspid regurgitation velocity >3.5m/s) (I C)
3. Pulmonary valve replacement in asymptomatic patients with severe pulmonary regurgitation and/or stenosis when at least one of the following criteria is present:
• Decrease in objective exercise capacity
• Progressive right ventricular dilation or systolic dysfunction
• Progressive tricuspid regurgitation (at least moderate)
• Right ventricular outflow tract obstruction with right ventricular systolic pressure >80 mmHg (tricuspid regurgitation velocity >4.3 m/s)
• Sustained atrial/ventricular arrhythmias (IIaC)
4. VSD closure in patients with residual VSD and significant left ventricular volume overload or if the patient is undergoing pulmonary valve surgery (IIaC)
References
1. ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957
2. Oosterhof T, van Straten A, Vliegen HWet al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation (2007);116:545–551.
Expert’s conclusions:
The patient was qualified first for percutaneous angioplasty of left pulmonary branch stenosis. In a few months surgical pulmonary valve replacement and tricuspid valve repair should be considered.
Authors:
Tomkiewicz-Pająk L. MD, PhD1, Podolec J. MD3, Kopeć G. MD, PhD2, Drabik L. MD1, Assoc. Prof. Olszowska M. MD, PhD2, Miszalski-Jamka T. MD, PhD2, Assoc. Prof. Kostkiewicz M. MD, PhD1, Tyrka A. MD1
Experts:
Prof. Skalski J.MD, PhD4, Kordon Z.MD, PhD5, Kopec G.MD, PhD1
1 Department of Cardiac and Vascular Disease, 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 Department of Pediatric Cardiology, Polish–American Children’s Hospital, Krakow, Poland
