Obstructed hemianomalous pulmonary venous drainage: is intervention necessary? Kevin S. Roman, Anthony P. Salmon and Joseph J.

Vettukattil Ann Thorac Surg 2002;74:1238-1240

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The Annals of Thoracic Surgery is the official journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association. Copyright 2002 by The Society of Thoracic Surgeons. Print ISSN: 0003-4975; eISSN: 1552-6259.

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CASE REPORT ROMAN ET AL SELF-RESOLUTION OF SCIMITAR SYNDROME

Ann Thorac Surg 2002;74:1238 40

conduction bundle and extended into right ventricle. Residual shunt as a result of the detachment of the patch was found at the superiorposterior corner of the septum and was closed with an oval-shaped pericardial patch. After resection of the right ventricular free wall muscle, a monocusped patch was designed for the enlargement of the right ventricular outow tract. The patient is working full-time work since the second operation.

Patient 2
Patient 2, a 2-year-old boy was admitted to Jikei University hospital with the diagnosis of double inlet left ventricle, a left-anterior rudimentary right ventricle, two well-functioning atrioventricular valves, and a large subaortic ventricular septal defect. This patient underwent a pulmonary artery banding at the age of 4 months. He had mild cyanosis when crying. The electrocardiogram showed rst degree atrioventricular block. Catheterization demonstrated a mild pulmonary hypertension, pulmonary vascular resistance of 2.2 Wood units, and a mild subaortic stenosis with a pressure gradient of 25 mm Hg at the ventricular septal defect. Echocardiography showed a restrictive ow across the ventricular septal defect and a left ventricular end-diastolic volume of 134% of normal value. On October 1995, ventricular septal defect was enlarged posteroinferiorly under cardiopulmonary bypass. Ventricular septation using a pericardial patch with Dacron reinforcement was carried out. Because of the relatively small ventricle, the septation patch was sutured to divide the main chamber in an attempt to make right ventricle-to-left ventricle volume ratio of 30/70. The patient was uneventfully weaned off the cardiopulmonary bypass in sinus rhythm. Catheterizations were done at 3 months and 3 years after the operation. Left ventricular end-diastolic volume was 170% at 3 months, 201% at 3 years after operation. The right ventricular end-diastolic volume was 101% at 3 months and 83% at 3 years. Although the right ventricular pressure was slightly elevated at 38/5 mm Hg 3 years after the operation, the patients subsequent course has been satisfactory.

Patient 2 had a suboptimal left ventricle for ventricular septation. Kurosawa and associates [5] reported that more than 170% of the normal value of the ventricle is necessary for septation. Preoperatively, the ventricle was estimated at 134% of normal. The patch was fashioned to divide a single ventricle into a relatively small right ventricle and a relatively large left ventricle. It is also essential that the septation patch be small to avoid systolic deviation to the right ventricle. The right ventricle-to-left ventricle volume ratio was 101/170 mm Hg 3 months after septation, which decreased to 83/200 mm Hg 3 years later. This procedure may contribute to good hemodynamics.

References
1. McGoon DC, Danielson GK, Ritter DG, Wallace RB, Maloney JD, Marcelletti C. Correction of the univentricular heart having two atrioventricular valves. J Thorac Cardiovasc Surg 1977;74:21826. 2. Ebert PA. Staged partitioning of single ventricle. J Thorac Cardiovasc Surg 1984;88:90813. 3. Kawashima Y, Mori T, Matsuda H, Miyamoto K, Kozuda T, Manabe H. Intraventricular repair of single ventricle associated with transposition of the great arteries. J Thorac Cardiovasc Surg 1976;72:217. 4. Arai T, Sakakibara S, Ando M, Takao A. Intracardiac repair for single or common ventricle, creation of a straight articial septum. Singapore Med J 1973;14:1879. 5. Kurosawa H, Imai Y, Fukuchi S, et al. Septation and Fontan repair of univentricular atrioventricular connection. J Thorac Cardiovasc Surg 1990;99:3149.

Obstructed Hemianomalous Pulmonary Venous Drainage: Is Intervention Necessary?

Kevin S. Roman, MRCP(UK), Anthony P. Salmon, MD, and Joseph J. Vettukattil, MD
Department of Paediatric Cardiology, Wessex Cardiothoracic Unit, Southampton General Hospital, Southampton, United Kingdom

Comment
There have been numerous accounts in the literature describing encouraging results in patients with doubleinlet left ventricle [2, 3]. But little is known about longterm survivors after this procedure. Arai and colleagues [4] reported a successful ventricular septation in 1973. This patient, who is now 37-year-old, has been a full-time worker and, to our knowledge, is considered to be the longest survivor. The patients we studied were operated on in 1971 and in 1995, respectively. During the two decades between the two patients, many aspects of cardiac surgery have dramatically progressed. Surgical technique, anesthesia, myocardial protection, and postoperative management have become more sophisticated. Despite the lack of conventional management, septation provided excellent long-term quality of life for Patient 1. Arai and colleagues determined that the size of the septation patch should be decided in the ventricular systolic phase [4].
2002 by The Society of Thoracic Surgeons Published by Elsevier Science Inc

Obstructed infradiaphragmatic hemianomalous pulmonary venous drainage is associated with high mortality and morbidity, and treatment is best tailored to the individual patient. Resolution of an obstructed scimitar vein through collateralization has rarely been reported. We report two such cases and review the literature. (Ann Thorac Surg 2002;74:1238 40) 2002 by The Society of Thoracic Surgeons ight hemianomalous pulmonary venous drainage is a rare abnormality characterized by a descending vein that enters the inferior vena cava (IVC) just below or above the diaphragm. This malformation, termed scimitar syndrome, is associated with other abnormalities
Accepted for publication May 1, 2002. Address reprint requests to Dr Vettukattil, Department of Paediatric Cardiology, Southampton General Hospital, Tremona Rd, Southampton SO166YD, UK; e-mail: joseph.vettukatti@lycos.com.

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Ann Thorac Surg 2002;74:1238 40

CASE REPORT ROMAN ET AL SELF-RESOLUTION OF SCIMITAR SYNDROME

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including hypoplasia of the right lung, cardiac dextroposition, bronchial anomalies, and anomalous arterial supply to the right lung. The interatrial septum is usually intact, and the relative pulmonary vascular resistance and the compliance of the atrial chambers determine the shunt size. Unobstructed hemianomalous drainage usually remains asymptomatic in childhood but can cause some dyspnea with exercise and can be seen with pneumonia. Obstructed scimitar drainage, however, usually causes symptoms and if left untreated, can lead to pulmonary hypertension. Scimitar syndrome is rarely associated with supraventricular tachycardia [1].

Case Reports Patient 1

A 14-month-old infant was seen with a 3-day history of vomiting. His birth was normal, and he had no medical history. He was tachypneic (respiratory rate, 50 breaths/ min) and tachycardic (heart rate, 240 beats/min) with an electrocardiogram showing preexcitation after cardioversion. A chest radiograph revealed cardiac dextroposition with hypoplasia of the right lung. An echocardiogram showed an intact interatrial septum and a dilated right atrium. A right-sided descending vein was identied entering the IVC, and normal left-sided pulmonary venous drainage was conrmed. When the patient was 15 months old, catheterization conrmed right-sided obstructive scimitar venous drainage. The ratio of pulmonary blood ow to systemic blood ow was 1.35:1, and the mean pulmonary artery pressure was 18 mm Hg. The mean right pulmonary capillary wedge pressure was 16 mm Hg compared with the mean left wedge pressure of 10 mm Hg, an indication of obstruction. The right upper and right lower pulmonary veins drained through a single descending vein to enter the IVC, and a small collateral arose from the descending vein to drain into the left atrium (Fig IA). The descending vein narrowed as it entered the IVC, and the IVC was partially obstructed at its junction with the right atrium. Blood ow from the IVC emptied into the right atrium by way of hepatic venous collaterals. Angiography excluded an abnormal arterial supply to the right lung. As the patient remained asymptomatic, palliative balloon angioplasty of the IVC and anomalous vein was planned. Catheterization at 19 months revealed that the IVC was completely occluded, hepatic venous collaterals maintained IVC drainage to the right atrium. The mean pressures were equal in both branch pulmonary arteries (13 mm Hg). Angiography showed that the descending vein now drained through a tortuous collateral vessel into the left atrium (LA), a nding explaining the absence of symptoms and hemodynamics (Fig 1B).

Fig 1. Patient 1. (A) Inferior vena cava (IVC) injection showing scimitar syndrome and partial obstruction of IVC (OI). (B) Wedge angiography of right pulmonary artery at second catheterization (anteroposterior projection). (CV collateral vessel; DV descending vein; LA left atrium; RA right atrium.)

Patient 2
A 5-month-old infant was seen with a murmur and recurrent chest infections. She appeared well with saturations of 95% but had mild subcostal recession. Other cardiac ndings were normal. An electrocardiogram re-

vealed poor R-wave progression, and a chest radiograph showed dextroposition of the heart and a visible scimitar vein. An echocardiogram conrmed a 10-mm secundum atrial septal defect. A right-sided descending vein entered the IVC below the diaphragm.

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CASE REPORT GARRETT ET AL HUMORAL REJECTION TREATED WITH RITUXIMAB

Ann Thorac Surg 2002;74:1240 2

Hemianomalous drainage was shown angiographically, and oximetry suggested a left-to-right shunt of 2:1. An arterial supply from the descending aorta to the posterior basal segment of the right lower lobe was identied. The pulmonary artery pressure was normal (mean, 16 value, mm Hg). Surgical intervention was planned in view of the recurrent chest infections and the atrial septal defect. When the patient was 20 months old, a surgical bafe was placed the IVC to redirect the descending vein through the atrial septal defect to the LA. The arterial supply to the sequestered lobe was ligated. The patients symptoms improved, and recovery was uneventful. Three years later, elective angiography demonstrated complete bafe occlusion. Pulmonary venous return now reached the LA by way of a tortuous collateral vessel. The mean pulmonary artery pressure was 14 mm Hg, and no intracardiac shunt was identied. The patient remains very well with good energy levels.

Resolution of an obstructed scimitar vein through collateralization has rarely been reported. The cases of these 2 patients demonstrate that collateralization can occur spontaneously or postoperatively. Coil embolization of the arterial supply to the sequestered lobe with the descending vein, left intact should be considered when chest infections are the only symptom. These cases support a conservative approach with close monitoring of pulmonary artery pressures in relatively well patients with obstructed hemianomalous pulmonary venous drainage. Careful monitoring with chest roentgenograms, electrocardiograms, and echocardiograms for estimating right ventricular pressures and recatheterizations to conrm protection of pulmonary vasculature are our current approach to patients with this complex condition.

References
1. Bruinsma GJ, Wever EF, Robles de Medina EO. Radiofrequency catheter ablation of AV nodal re-entrant tachycardia in scimitar syndrome. Int J Cardiol 1997;58:3057. 2. Sanger PW, Taylor FH, Charlotte FR. The scimitar syndrome: diagnosis and treatment. Arch Surg 1963;86:8491. 3. Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years experience and results of repair. J Thorac Cardiovasc Surg 1996;112:161 8. 4. Dupuis C, Charaf LAC, Breviere G, Abou P. Infantile form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 1993;71:132630. 5. Mas C, Goh TH, Wilkinson JL. New interventional therapeutic approach for dual drainage of the scimitar vein. Catheter Cardiovasc Interv 2000;51:1925.

Comment
The management of scimitar syndrome is complex, and the indications for intervention are a large shunt resulting in pulmonary hypertension and heart failure or lobar sequestration or recurrent pneumonia [2]. Scimitar syndrome presenting in infancy has been associated with higher mortality and morbidity than the same diagnosis is made later [3, 4]. Intervention involves coil embolization or ligation of the arterial supply to the sequestered lobe and surgical redirection of the anomalous vein to the LA. After operation, pulmonary vein stenosis and abnormal blood ow to the right lung remain concerns. Najm and colleagues [3] reported a 47% incidence of pulmonary vein stenosis after an atrial bafe operation and found that pulmonary blood ow to the right lung was seldom normal after surgical repair. The case of patient 1 demonstrates obstructed scimitar drainage with spontaneous resolution. The elevated right pulmonary capillary wedge pressure conrmed obstruction within the anomalous pulmonary venous connection. A small supradiaphragmatic collateral vessel from the scimitar vein draining to the LA allowed the patient to remain asymptomatic despite complete occlusion of the infradiaphragmatic segment of the scimitar vein. The right and left pulmonary artery wedge pressures were no longer discrepant and had normalized. Mas and colleagues [5] reported a similar case but with dual drainage of the scimitar vein to the LA. They performed device occlusion of the lower scimitar vein, thereby leaving sole drainage by way of a second scimitar vein. The case of patient 2 demonstrates the potential longterm problems associated with surgical repair using an intracardiac bafe. Despite close follow-up and an absence of symptoms, progressive bafe obstruction occurred. A collateral vessel developed (as in patient 1) despite complete obstruction of the surgical pathway. In this patient, surgical intervention did not contribute to the nal outcome, and the same result might have been achieved by occlusion of the arterial supply alone.
2002 by The Society of Thoracic Surgeons Published by Elsevier Science Inc

Treatment of Humoral Rejection With Rituximab

H. Edward Garrett, Jr, MD, Kenneth Groshart, MD, Denise Duvall-Seaman, RN, BSN, Deena Combs, RN, BSN, and Rita Suggs, RN, BSN
Division of Cardiothoracic Surgery, University of Tennessee, Memphis, and Baptist Hospital/University of Tennessee Heart Transplant Program, Memphis, Tennessee

Humoral vascular rejection is a B cellmediated production of immunoglobulin G antibody against the transplanted organ. Available treatments of vascular rejection offer limited success, and chronic manifestations of vascular rejection require retransplantation. On the basis of the mechanism of action of rituximab, we successfully treated 1 patient with vascular rejection refractory to plasmapheresis with this drug without major toxicity. (Ann Thorac Surg 2002;74:1240 2) 2002 by The Society of Thoracic Surgeons

Accepted for publication May 1, 2002. Address reprint requests to Dr Garrett, 80 Humphreys Blvd, Suite 300, Memphis, TN 38120.

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Obstructed hemianomalous pulmonary venous drainage: is intervention necessary? Kevin S. Roman, Anthony P. Salmon and Joseph J. Vettukattil Ann Thorac Surg 2002;74:1238-1240
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