Ann Thorac Surg 2003;76:2089-2091
© 2003 The Society of Thoracic Surgeons
Case report
Restrictive interatrial communication in hypoplastic left heart syndrome after modified Fontan repair
Hassan W. Nemeh, MDa,
Sushma Reddy, MDb,
Henry L. Walters, III, MDa,
Mehdi Hakimi, MDa,
Robert D. Ross, MDb*
a Cardiovascular Surgery and Cardiology, Detroit, Michigan, USA
b Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan, USA
Accepted for publication April 9, 2003.
* Address reprint requests to Dr Ross, Department of Cardiology, Children's Hospital of Michigan, 3901 Beaubien St, Detroit, MI 48201, USA
e-mail: rross{at}dmc.org
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Abstract
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The occurrence of pulmonary venous obstruction after total cavopulmonary connection with intraatrial lateral tunnel is a rare occurrence. We present two cases of hypoplastic left heart syndrome with restrictive interatrial communication presenting late after this type of modified Fontan repair. This occurred even after complete excision of the atrial septum at the time of Stage 1 Norwood in both cases. A novel approach to this problem of resecting the roof of the coronary sinus was utilized to enlarge the interatrial communication.
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Introduction
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In 1980, Norwood and associates [1] described their initial experience with surgical palliation of patients with hypoplastic left heart syndrome [HLHS]. There has since been an improved outlook for these patients with the staged reconstruction. Complications after Fontan repair have included effusions, arrhythmias, protein losing enteropathy, thrombotic events, cirrhosis, and pulmonary venous obstruction [2–4]. Here, we report two cases of pulmonary venous obstruction due to an acquired restriction of the atrial septal defect that presented as late complications of total cavopulmonary connection with intraatrial lateral tunnel.
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Case reports
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Case 1
A.W., a term infant, was diagnosed with HLHS. He underwent a Norwood palliation at five days followed by a Hemi-Fontan at six months. Cardiac catheterization done in anticipation of stage 3 surgery showed no pressure gradient between the left and right atrium. The modified Fontan was completed at 27 months, after which he was treated for recurrent pleural effusions and mild hepatomegaly. The child did well for a year, during which time an echocardiogram showed minimal flow acceleration across the interatrial communication (IAC).
He presented at 4 1/2 years with progressive abdominal distension and occasional duskiness. He had mild central cyanosis, tachycardia, abdominal distension, and hepatomegaly. Chest roentgenogram showed mildly prominent proximal pulmonary arteries and an echocardiogram (Fig 1)
suggested a restrictive interatrial communication with the lateral tunnel abutting the posterior margin of the IAC. Cardiac catheterization confirmed this obstruction with a gradient of 8 mm Hg across the IAC and an elevated pulmonary artery mean pressure of 16 mm Hg.
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Fig 1. Subcostal echocardiograph from case 1 demonstrating a small interatrial communication (arrow). The asterisk is within the lateral tunnel. (LA = left atrium; RV = right ventricle.)
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Case 2
E.W., a term infant, was diagnosed with HLHS and underwent a first stage Norwood palliation at six days, a Hemi-Fontan at 8 months, followed by a modified Fontan repair at 20 months. The patient did well for three months after which she developed recurrent pleural effusions. This was managed with drainage, diuretics, and chemical pleurodesis. When the effusion recurred, an echocardiogram suggested a restrictive interatrial communication, and cardiac catheterization confirmed a gradient of 9 mm Hg between the mean pulmonary wedge pressure and the right ventricular end diastolic pressure. Angiography demonstrated a narrowing at the level of the IAC.
Both cases were operated on for surgical relief of the pulmonary venous obstruction at the level of the atrial septum. The operative findings were similar in each and revealed significant fibrosis at the edges of the IAC with contracture of tissue and shrinkage in the size of the opening. There was no evidence of compression of the pulmonary veins or encroachment by the lateral tunnel on the IAC. Initially, resection of the residual margins of the interatrial communication was performed. When this did not adequately relieve the obstruction, the coronary sinus was widely unroofed into the left atrium to provide an unrestricted communication (Fig 2)
in both patients. Each had an uneventful recovery with complete resolution of their symptoms.
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Fig 2. (A) Surgical view of the opened right atrium showing the small interatrial communication in the midseptum. (B) Unroofing of the coronary sinus with extension of the incision up to the interatrial communication. (C) Afterwards, the interatrial communication is widely patent with visualization of the left pulmonary veins.
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Comment
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Our patients developed right heart failure and pleural effusions due to progressive restriction of the IAC 2 yrs and 9 m after the modified Fontan operation. A new surgical approach for this was to widely unroof the coronary sinus into the left atrium to provide an unrestricted communication. This technique was first utilized for repairing total anomalous pulmonary venous return to the coronary sinus [5].
Restrictive IAC in the HLHS has been described more commonly before the second stage of cavopulmonary anastomosis with an incidence of 3% to 4% [6]. Infants born with restrictive atrial septal defects may be more prone to developing late restriction, although neither of our patients had this at birth. Restrictive IAC has rarely been reported after the completion of the Fontan procedure for HLHS [2, 7]. Table 1
summarizes all reported cases of restrictive IAC in HLHS after modified Fontan repair. The cause of the progressive obstruction is not known, but may be due to hyperplasia or scarring of the edges of the septal defect.
Our two cases demonstrate that restriction of the atrial defect may occur as a late complication any time after the modified Fontan repair and should be looked for in the face of clinical signs of heart failure or recurrent pleural effusions. The addition of unroofing the coronary sinus to the resection of the remaining atrial septum is a new modification to treat this problem, and provides complete relief of the obstruction.
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References
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- Norwood W.I., Kirklin J.K., Sanders S.P. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol 1980;45(1):87-91.[Medline]
- Fogel M.A., Chin A.J. Imaging of pulmonary venous pathway obstruction in patients after the modified Fontan procedure. J Am Coll Cardiol 1992;20(1):181-190.[Abstract]
- Berman W., Jr, Fripp R.R., Yabek S.M. Late-onset pulmonary venous pathway obstruction after Fontan operation: presentation masquerading as intra-atrial baffle leakage. Pediatr Cardiol 1997;18(1):49-51.[Medline]
- Kreutzer J., Keane J.F., Lock J.E., et al. Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg 1996;111(6):1169-1176.[Abstract/Free Full Text]
- Cooley D.A., Hallman G.L., Leachman R.D. Total anomalous pulmonary venous drainage. Correction with the use of cardiopulmonary bypass in 62 cases. J Thorac Cardiovasc Surg 1966;51:88-102.[Medline]
- Mahle W.T., Rychik J., Gaynor J.W., Spray T.L., Rome J.J. Restrictive interatrial communication after reconstructive surgery for hypoplastic left heart syndrome. Am J Cardiol 2001;88(12):1454-1457.[Medline]
- Riexinger M., Galm C., Trotter A., Lang D. Restrictive interatrial communication with protein-losing enteropathy and coagulopathy in hypoplastic left heart syndrome after Norwood palliation. Pediatr Cardiol 2002;23(1):84-86.[Medline]
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Abstract
Introduction
