Female patient after correction of tetralogy of Fallot with severe pulmonary regurgitation and significant left-to-right shunt at the ventricular septal level (RCD code: IV-5A2)

Leszek Drabik, Lidia Tomkiewicz‑Pająk, Tomasz Miszalski-Jamka, Jacek Pająk, Bogusław Kapelak, Piotr Podolec

Full Text:



Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease after 1 year of age. Less than 5% of all patients with uncorrected ToF live beyond the age of 40 years. Prognosis after surgical correction is good with a 32-year survival of 86% of the cases. Predictors of long-term mortality include older age at operation and postoperative right-to-left ventricular peak systolic pressure ratio of 0.5 or higher [1]. Surgery is performed to close ventricular septal defect (VSD) and relieve right ventricular outflow tract obstruction(RVOTO). Adult patients with surgical repair of tetralogy of Fallot in history often develop complications, which include severe pulmonary regurgitation, RVOTO, right ventricular dilation and dysfunction, residual VSD, aortic root dilation with aortic regurgitation, left ventricular dysfunction, endocarditis, atrial and ventricular tachycardia, and sudden cardiac death. We present a case of a female patient with several long-term complications after ToF repair. JRCD 2014; 1 (7): 24–28


Tetralogy of Fallot, residual ventricular septal defect, pulmonary regurgitation


Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993; 329: 593–599.

Frigiola A, Redington AN, Cullen S, Vogel M. Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot. Circulation 2004; 110: 153–157.

Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000; 356: 975–981.

Silversides CK, Veldtman GR, Crossin J, et al. Pressure Halftime predicts hemodynamically significant pulmonary regurgitation in adult patients with repaired tetralogy of Fallot. J Am Soc Echocardiogr 2003; 16: 1057–1062.

Lancellotti P, Tribouilloy C, Hagendorff A, et al. European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 1: aortic and pulmonary regurgitation (native valve disease) Eur J Echocardiogr 2010; 11: 223–244.

Puchalski MD, Askovich B, Sower CT, et al. Pulmonary regurgitation: determining severity by echocardiography and magnetic resonance imaging. Cong Heart Dis 2008; 3: 168–175.

Task Force on the Management of Grown Up Congenital Heart Disease, European Society of Cardiology; ESC Committee for Practice Guidelines. Management of grown up congenital heart disease. Eur Heart J. 2003; 24: 1035–1084.

Vliegen HW, van Straten A, de Roos A, et al. Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of Fallot. Circulation 2002; 106: 1703–1707.

Oosterhof T, van Straten A, Vliegen HW, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 2007; 116: 545–551.

Lee C, Park CS, Lee CH, et al. Durability of bioprosthetic valves in the pulmonary position: long-term follow-up of 181 implants in patients with congenital heart disease. J Thorac Cardiovasc Surg. 2011; 142: 351–358.

Ovcina I, Knez I, Curcic P, et al. Pulmonary valve replacement with mechanical prostheses in re-do Fallot patients. Interact Cardiovasc Thorac Surg. 2011; 12: 987–991.

Lurz P, Nordmeyer J, Giardini A, et al. Early Versus Late Functional Outcome After Successful Percutaneous Pulmonary Valve Implantation: Are the Acute Effects of Altered Right Ventricular Loading All We Can Expect? J. Am. Coll. Cardiol. 2011; 57; 724–731.

Eicken A, Ewert P, Hager A, et al. Percutaneous pulmonary valve implantation: two-centre experience with more than 100 patients. Eur Heart J 2011; 32: 1260–1265.

Vahanian A, Baumgartner H, Bax J, et al. Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J. 2007; 28: 230–268.

Hanna BM, El-Hewala AA, Gruber PJ, et al. Predictive value of intraoperative diagnosis of residual ventricular septal defects by transesophageal echocardiography. Ann Thorac Surg. 2010; 89: 1233–1237.

DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no7.141


  • »
  • »
  • »
  • »
  • »
  • »
  • »
  • »
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659
e-mail: softq@softq.pl