Background
Truncus arteriosus communis (TAC) is a rare congenital heart disease appearing in 0.034 to 0.56 per 1,000 newborns and representing 1.4% to 2.8% of all cases of congenital heart diseases. In this anomaly, a single arterial trunk arises from the heart, overrides the interventricular septum, and supplies systemic, pulmonary, and coronary circulations. Without surgical treatment, 80% of patients die within the first year of life, usually in early infancy. Repair of TAC during the neonatal and early infant period has become standard practice in many centers, with very good results. The authors presents a case of patient after Common Trunk type I operation.
Case description
In this 21-year-old Caucasian male the Common Trunk type I was diagnosed on the second day of life based on cardiological consultation at the Department of Cardiology, Medical Academy Krakow. The defect was repaired on August 6th, 1990 by implanting Homograft No 9 pulmonary NHH and Dacron conduit No 12 in surgical procedure. In 1993 reoperation was performed due to stricture of pulmonary Homograft. Homograft No 19 pulmonary NHH and Dacron conduit No 22 were implanted surgically. No complications were observed after both procedures.
The patient was referred to our Centre by his GP in May 2011 with suspicion of pulmonary Homograft stricture based on echocardiographic examination performed prior to admission. On admission he is hemodynamically stable with no signs of peripheral or pulmonary oedema. His heart rate is 75 beats/ minute and blood pressure 135/80 mmHg. He is considered to be in NYHA class I. He complains of minor exercise capacity reduction. There is no family history of congenital heart defects, no history of smoking and good socioeconomic living conditions. Additionally he suffers from bronchial asthma and he underwent pyloroplasty in 1990 due to inborn pylorus stenosis. He also complains of recurrent migraine headaches. On physical examination no abnormalities were detected. Biochemical blood analysis shows normal values of complete blood count, no signs of kidneys or liver dysfunction.
On transthoracic echo organized calcification of the Homograft and stricture of the pulmonary conduit is observed. The pressure gradient in right ventricular outflow tract is 99.9/66 mmHg. In 24-hr ECG monitoring no abnormalities are detected. Exercise capacity is considered to be within the reference range after accomplishing cardiopulmonary exercise test at 15 min 2 s with the load of 11.7 METs. No chest pain or any arrhythmias or ST segment deviations were observed during the test. Oxygen consumption is 23.4 ml/kg/min.
CT heart study shows calcified Homograft and stenosis of Dacron pulmonary conduit. Right heart catheterization reveals high right ventricular pressure (100 mmHg) and elevated pressure in the pulmonary graft (100 mmHg).
Guidelines
ESC guidelines for the management of grown-up congenital heart disease published in 2010 provide indications for intervention in patients with right ventricular to pulmonary artery conduits. Based on these recommendations asymptomatic patients with severe right ventricular outflow tract obstruction (RVOTO) and/or severe pulmonary regurgitation (PR) should be considered for surgery when at least one of the following criteria is present (IIa C):
• Decrease in exercise capacity (CPET)
• Progressive RV dilation
• Progressive RV systolic dysfunction
• Progressive TR (at least moderate)
• RV systolic pressure >80 mmHg (TR velocity >4.3 m/s)
• Sustained atrial/ventricular arrhythmias
Also, diagnostic workup for patients with right ventricular to pulmonary artery conduits is enclosed in the ESC guidelines. Echocardiography is the first-line diagnostic tool providing size and function of both ventricles, PR, TR, and associated lesions. Gradients across the conduit may be difficult to measure and not reliable. RV pressure derived from TR velocity should be used to assess conduit stenosis. CMR and CT may be required to image the conduit (level of stenosis), PA, and coronary artery anatomy, for the assessment of the RV and severity of PR. Catheterization with haemodynamic assessment is always required if intervention is considered. Angiography provides information on the level of stenosis, peripheral PA stenoses, and coronary anatomy (anomalies/abnormal course).
Longitudinal data of the image of the homograft and conduit are more important for timing of reintervention than single measurements. Regular follow-up in a specialized GUCH centre is recommended at least every 12 months.
References
1. Moller JH. Prevalence and incidence of cardiac malformations In: Moller JH, editor. Surgery of congenital heart disease, Pediatric Cardiac Care Consortium 1984–1995. Philadelphia: Futura; 1998. pp. 19-26.
2. Corno AF. Congenital heart defects. Decision making for surgery. Vol 2. Darmstadt, Germany: Steinkopf Verlag; 2004. pp. 71-81.
3. ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957.
Experts’ comments:
1. Janusz Skalski, MD, PhD: Surgical approach is not recommended at this point.
2. Jacek Pająk, MD, PhD: Surgical procedure can be considered in case of developing new symptoms.
3. Maria Olszowska MD, PhD: Patient needs regular follow-up and optimal medical treatment. Reoperation is not recommended now.
4. Tomkiewicz- Pająk L MD, PhD: Echocardiography is the first-line diagnostic tool providing size and function of both ventricles, PR, TR, and associated lesions. Gradients across the conduit may be difficult to measure and not reliable. RV pressure derived from TR velocity should be used to assess conduit stenosis. CMR and CT may be required to image the conduit (level of stenosis), PA, and coronary artery anatomy, for the assessment of the RV and severity of PR.
Conclusions:
Patient is qualified for optimal medical treatment and regular follow-up. TTE with careful assessment of RV function, RV systolic pressure (conduit gradient) and TR is required at 3 months period. Periodical CPET and Holter examination is required. In case of new symptoms arising and worsening of patient’s clinical status reevaluation of indications for surgery is necessary.
Authors:
Komar M.3 , Wilkołek P.3 , Kopeć G.3 , Sobień B.3 , Stępniewski J.3 , Tyrka A.3, Widlińska B.3, Podolec P.3
Experts:
Janusz Skalski1, Jacek Pająk2, Olszowska M.3, Tomkiewicz- Pająk L.3,
1 Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
2 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
3 Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
