(IV-5A.2) Patient with significant pulmonary valve regurgitation after Fallot operation Lidia Tomkiewicz-Pająk MD. PhD., Maria Olszowska MD. PhD., Prof. Piotr Podolec MD. PhD.

Background
Tetralogy of Fallot (ToF) is the 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 present complications. They include: 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 patient after ToF repair with complex complications.

Case presentation
A 19-year-old man with Tetralogy of Fallot with pulmonary atesia, hypoplasia of pulmonary arteties. At the age of 5 years he was operated right ventricle – pulmonary artery coduit was performed. At the age of 6,5 years the reoperation of right ventricle oulet truct reconstruction was performed with angioplasty of pulmonary braches with stents implantation. At the age of 7 and 13 years redilatatio of stents in pulmonary arteries were performed. The patient had been systematically carried by the cardiologist. He was asymptomatic with good exercise tolerance and he had been clinically stable since 2000 . Aditional he had a history of scoliosis, double right kidney and mental handicap.
Laboratory studies have found: E – 5040000 Hg- 14,8 g/dl, Ht- 44%, ASPAT- 30 U/L, ALAT- 33 U/L, Protein – 76,8g/L, pBNP – 299 pg/ml
Holter monitoring revealed sinus rhythm with single ventricle extrasystolies. Echocardiography showed impaired function of rigt ventricle, major pulmonary valve regurgitation, major tricuspid valve regurgitation, preserved right ventricle diastolic function – TAPSE 28 mm, S” 14 cm/sek, stenosis of right pulmonary valve with gradient 45/33 mmHg. MR showed impaired function of right ventricle and severe stenosis of left pulmonary branch.

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 ventricle 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 ventricle 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 ventricle volume overload or if the patient is undergoing pulmonary valve surgery (IIaC)

Expert’s comments:(Written authorization required from each expert)

1.Prof. Janusz Skalski, MD, PhD.²
Asymptomatic patient with Tetralogy of Fallot after many steps of correction with impaired right ventricle systolic function, significant pulmonary valve regurgitation, with right pulmonary artery stenosis. The patient should be qualified first for percutaneus angioplasty of right pulmonary branch stenosis.

2. Jacek Pająk MD, PhD³
Patient with Tetralogy of Fallot after many steps of correction with significant pulmonary and tricuspid valves regurgitation and significantly increased end diastolic right ventricle volume needs pulmonary valve implantation. Tricuspid valve ring implantation also should be consider. Right pulmonary artery gradient should be mesure and percutaneus angioplasty of right pulmonary branch stenosis should be considered.

3. Wojciech Płazak, MD, PhD¹
Late complications in the patients operated due to Tetralogy of Fallot consists a frequent problem. In this case we see significant pulmonary valve regurgitation, with right pulmonary artery stenosis. The function of right ventricle seems, however, be preserved (TAPSE = 28 mm, S’ = 14 cm/s). I would suggest first the percutaneus angioplasty of right pulmonary branch stenosis and the reevaluation of the hemodynamics after this procedure.

Expert’s conclusions:
The patient was qualified first for percutaneus angioplasty of right pulmonary branch stenosis. After a few mouths surgical pulmonary valve replacement and tricuspid valve repair should be considered.

Authors:
Lidia Tomkiewicz-PająkMD. PhD.¹, Maria Olszowska MD. PhD.¹, Prof. Piotr Podolec MD. PhD.¹

Experts:
Prof. Janusz Skalski, MD, PhD.², Jacek Pająk MD, PhD³, Wojciech Płazak, MD, PhD¹

¹Department of Heart and Vascular Disease, John Paul II Hospital, Kraków, Poland
²Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
³Independent 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