Ann Thorac Surg 2005;80:e17-e19
© 2005 The Society of Thoracic Surgeons
Case report
Staged Correction of Double Discordance, Situs Inversus, and Absence of True Pulmonary Arteries
Federico Brunelli, MD
a
,
*
,
Andrea Amaducci, MD
a
,
Giordano Tasca, MD
a
,
Zen Mhagna, MD
a
,
Bruno Amari, MD
b
a Division of Cardiac Surgery, Brescia, Italy
b Division of Cardiac Anesthesia and Postoperative Intensive Care, Private Non-Profit Hospital Poliambulanza, Brescia, Italy
Accepted for publication June 7, 2005.
* Address correspondence to Dr Brunelli, Division of Cardiac Surgery, Private Non-Profit Hospital Poliambulanza, via Bissolati 57, Brescia, 25125 Italy (Email: cch-segreteria.poli{at}poliambulanza.it).
 |
Abstract
|
|---|
The case of a young boy with an extremely rare and complex cardiac malformation is described. Situs inversus, dextrocardia, double discordance with pulmonary atresia, absence of true pulmonary arteries, and systemico-pulmonary collateral arteries was the original diagnosis. Three preparatory operations were necessary to reconstruct and unifocalize the pulmonary vascular tree. At the age of 7 years complete correction with intraoperative stenting of the left neopulmonary artery was successfully performed. After more than 3 years from the definitive operation, the patient is alive with no physical limitations.
|
Introduction
|
|---|
Pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries associated with congenitally corrected transposition of the great arteries is an extremely rare disease entity. Staged unifocalization of the major aortopulmonary collateral arteries followed by double switch operation has been recently described in 1 patient [1].
We encountered a similar malformation combined with situs inversus, dextrocardia, and absence of true pulmonary arteries. The patient underwent three different palliative operations and successful physiological correction at the age of 7 years. The patient's neonatal diagnosis was situs inversus, dextrocardia, a discordant atrioventricular connection, a single aortic outlet from the morphologically right ventricle, pulmonary atresia with no true pulmonary arteries, and a wide inlet-type VSD. The angiographically defined anatomy of the pulmonary circulation consisted of a single, long collateral from the left-sided descending aorta supplying the right lung, with stenosis at the origin of each lobar branch, and two short collaterals (from the left subclavian artery and the distal thoracic aorta) supplying the upper and lower part of the left lung, respectively.
Because of a balanced circulation the patient was discharged from the hospital and came to us for observation at the age of 11 months for increasing cyanosis. Three preparatory operations were necessary before total correction was attained. The first operation consisted of a left thoracotomy with construction of a bovine pericardial roll, anastomosis of the major aortopulmonary collateral arteries to this neopulmonary artery, and a modified Blalock-Taussig shunt using a 5-mm polytetrafluoroethylene graft from the ipsilateral subclavian artery. The second operation at 2 years of age consisted of a right thoracotomy with transection of the right collateral, flap enlargement of the right hilum using collateral tissue, and a modified Blalock-Taussig shunt with a 5-mm polytetrafluoroethylene graft. The third operation at the age of 3 years consisted of a right re-thoracotomy, patch angioplasty of the inferior lobar artery, which appeared angiographically disconnected from the superior artery, and a midline sternotomy on cardiopulmonary bypass with the heart beating, with an interposition of a 12-mm polytetrafluoroethylene conduit between the right hilum and the left neopulmonary artery (transverse conduit), and replacement of the side shunts with a 6-mm polytetrafluoroethylene graft between the right innominate artery and the transverse conduit. The anastomosis between the transverse conduit and the pericardial roll was extended on the inferior aspect of the roll in order to enlarge a previously documented stenosis. The systemic oxygen saturation was maintained at 75% to 85% throughout these operations.
At the age of 7 (weight, 28 Kg), the patient was readmitted to our department because of cyanosis at rest and breathlessness on minimal effort. His hemoglobin level was 21.5 g/dL, hematocrit was 61.5%, and peripheral oxygen saturation was 70% on room air. An echocardiographic assessment revealed two normally functioning ventricles, an inlet type VSD unconnected to the aorta, and normally competent atrioventricular valves. A preoperative angiographic study demonstrated the good patency of the shunt and transverse conduit, good perfusion of the right lung, and a recurrent, severe 20-mm long stenosis in the proximal part of the left sided pericardial roll (Fig 1). As any attempt to enter the shunt caused life-threatening hypoxia, the intraoperative placement of an endovascular device was planned.
Re-sternotomy was performed and the heart was freed from adhesions. A 6-French introducer sheath was positioned through a small stab incision in the transverse conduit between the ascending aorta and the left superior vena cava. Stenting of the left neopulmonary artery was successfully performed under angiographic control as described elsewhere [2]. Subsequently cardiopulmonary bypass was established through aortic and bicaval cannulation. During cooling to 26°C, the central shunt was tied, the ascending aorta was cross-clamped, and cold blood cardioplegia was administered through the aortic root and was then repeated every 20 minutes. The option of an anatomic correction by means of a double switch operation was previously abandoned for the long distance between the VSD and the aorta. The morphologically left ventricle (LV) was opened cranially to an important diagonal branch of the left anterior descending coronary artery; the morphologically right atrium was also opened. The VSD was closed with a bovine pericardial patch. The atrial septal defect was directly sutured. An oval-shaped incision was made in the transverse conduit and a 19-mm Freestyle stentless aortic root bioprosthesis (Medtronic Heart Valves, Minneapolis, MN) was anastomosed to it. A bovine pericardial gusset was used to connect the prosthesis to the morphologically LV (Fig 2). During myocardial reperfusion, the atriotomy was closed and the aorta was unclamped. The heart started beating spontaneously in sinus rhythm. Weaning from cardiopulmonary bypass was easily obtained after rewarming. Cross clamping and cardiopulmonary bypass time were 114 and 152 minutes, respectively. The ratio between the pulmonary and systemic ventricular pressure was 0.8 just at the end of cardiopulmonary bypass, which decreased to 0.5 before chest closure.
The postoperative course was uneventful, and after 12 days of hospitalization the child was discharged home fully saturated on room air with an echocardiographic estimate of morphologically LV systolic pressure of 40 mm Hg. A further angiographic study that was electively performed 15 months later demonstrated a moderate re-stenosis across the stented portion of the left neopulmonary artery. Re-dilation was successfully performed with good angiographic results and no residual gradient. At last clinical control (ie, 3 years postoperatively), the child was fully saturated, asymptomatic, without cardiac medication and physical limitation, and weighed 35 kg. The echocardiogram showed two well functioning ventricles, no residual septal defect, no atrioventricular valve insufficiency, and a morphologically LV systolic pressure of 40 mm Hg.
|
Comment
|
|---|
The combination of pulmonary atresia with major aortopulmonary collateral arteries and congenitally corrected transposition of the great arteries is extremely rare. Moreover the association with situs inversus, dextrocardia, and absence of true pulmonary arteries has never been described in the surgical literature. A staged surgical approach seems to be the unique solution for complete correction of the cardiopulmonary malformation. Sides have to be approached sequentially to construct prosthetic neopulmonary arteries and unifocalize their blood supply [3]. The final step of the surgical strategy will be the intracardiac repair and the connection of the heart with the pulmonary arteries. Two options can be considered: (1) the anatomical correction that implies an atrial switch associated with an intracardiac re-routing of the LV to the aorta and an extracardiac connection of the right ventricle with the pulmonary arteries; (2) a physiologic correction that consists of a simple septation of the atria and the ventricles followed from a conduit interposition between the morphologically LV and the pulmonary arteries. In our patient, we chose the second solution for the following reasons: small volume of the atrial cavities and their posterior location behind the ventricular mass, long distance between the aorta and the VSD with tricuspid cordae interposition, and higher than normal pulmonary artery pressure due to the presence of peripheral pulmonary stenosis. The decision to treat the principal stenosis in the left pulmonary artery by implantation of an endovascular device under direct vision was certainly useful, as confirmed from the easy and uneventful weaning of the patient from cardiopulmonary by pass. The optimal function of both ventricles and atrioventricular valves at discharge from the hospital was reconfirmed at an echocardiographic examination performed at almost 3 years after the operation, which is an encouraging find for the future of this child. A final observation is the use of a small heterologous pericardial roll (constructed at 11 months of age to replace an absent left pulmonary artery that was repaired twice at operations 3 and 4) is patent without calcification and thrombosis 10 years later.
|
References
|
|---|
- Ando M, Duncan BW, Mee RBB. Anatomic correction for corrected transposition after pulmonary unifocalization Ann Thorac Surg 2003;75:1012-1014.[Abstract/Free Full Text]
- Brunelli F, Amaducci A, Danzi GB. Intra-operative stenting of a prosthetic left pulmonary artery stenosis under fluoroscopy J Invas Cardiol 2005;17:98-99.[Medline]
- Uemura H, Yagihara T, Kawashima Y, Yamashita K, Kamiya T. Intrapulmonary reconstruction of pulmonary arteries using a heterologous pericardial roll Ann Thorac Surg 1995;59:1464-1469.[Abstract/Free Full Text]
Top
Abstract
Introduction
