- Email: [email protected]
A case with heterotaxy reaching the Fontan procedure after unifocalizations
International Journal of Cardiology 120 (2007) e3 – e5 www.elsevier.com/locate/ijcard
Letter to the editor
A case with heterotaxy reaching the Fontan procedure after unifocalizations Tomohiro Tsunekawa a , Toru Ishizaka a , Hideki Uemura b , Koji Kagisaki a , Ikuo Hagino a , Toshikatsu Yagihara a,⁎ b
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan Department of Cardio-thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
Received 1 January 2007; accepted 17 February 2007 Available online 11 June 2007
Abstract One patient with major aorto-pulmonary collateral arteries and heterotaxy underwent staged unifocalizations, and eventually the staged Fontan completion. Subsequent to the bidirectional Glenn procedure, the left pulmonary artery was once thrombozed, but successfully treated. The progressively regurgitant common atrioventricular valve needed repair twice during the course. Pulmonary arterial pressure was 11 mm Hg 1 year after the Fontan procedure. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Heterotaxy; Fontan; Univentricular heart; Pulmonary arteries
In the rare setting of major aortopulmonary collateral arteries (MAPCAs) with heterotaxy, a few successful surgical cases have been reported for anatomic repair [1], or the Fontan procedure [2]. We herein add another patient. 1. Clinical summary A young man with right isomerism of the atrial appendage had common inlet right ventricle (apex pointing right). The aorta (left arch) arose from the dominant ventricle with pulmonary atresia. Two MAPCAs maintained the pulmonary circulation. The pulmonary artery (PA) had recognizable intra-pericardial portions. The atria possessed virtually no septum, receiving all the pulmonary veins unobstructed and the bilateral superior and the left-sided inferior caval veins. The first operation took place at another hospital when 6 months old. Through a left thoracotomy, a 4 mm expanded polytetrafluoroethylene (ePTFE) tube was interposed be-
⁎ Corresponding author. National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. Tel.: +81 6 6833 5012; fax: +81 6 6872 7486. E-mail address: [email protected] (T. Yagihara). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.02.052
tween the subclavian artery and the central PA. Intending to protect the pulmonary vasculature from the progressive hypertensive change, the left MAPCA origin was banded. These markedly improved segmental imbalance in lung perfusion (Fig. 1B and C). He was referred to us for contemplating strategies. At 18 months old, arterial oxygen saturation (SaO2) was 70%. Angiography illustrated a favorable anatomy for unifocalization (Fig. 1A). Pulmonary flow was augmented through a systemic-to-pulmonary shunt of a 5 mm ePTFE tube (right thoracotomy). The right MAPCA was directly implanted to the intra-pulmonary artery [3]. One year later, SaO2 was 79%, mean PA pressure as high as 51 mm Hg, and Qp/Qs value 1.0. At 7 years old, SaO2 75%, mean PA pressure 17 mm Hg, and Qp/Qs 0.7. The left shunt was renewed using a 5 mm ePTFE tube (repeated left thoracotomy). Concomitantly, the MAPCA previously banded was implanted to the central PA. Home oxygen inhalation therapy was started. During following 3 years, SaO2 and Qp/Qs stayed over 80% and 1.2, respectively. Mean PA pressure decreased from 36 to 13 mm Hg, while moderate atrioventricular regurgitation progressed.
e4
T. Tsunekawa et al. / International Journal of Cardiology 120 (2007) e3–e5
Fig. 1. Angiography after the first palliation (A). The right major aorto-pulmonary collateral artery (MAPCA) exhibited stenosis (wide arrow) at its proximal portion, while the left one had been banded at its origin (dotted arrow). The central pulmonary artery (PA) bridged the upper lobes, and possessed intrapulmonary communications to the segments supplied via collaterals (thin arrow). Scintigraphy before (B) and after (C) the initial palliation. Attenuated perfusion to segments other than the left lower lobe vastly improved.
At 10 years old, the bilateral bidirectional Glenn procedure was carried out maintaining an additional flow to the lungs via a 5 mm ePTFE central shunt. The common atrioventricular valve was annulo-plicated by the so-called ‘figure-of-eight’ method. Fourteen months later, the left PA was found thrombosed. Moderate atrioventricular regurgitation recurred. Thrombi were surgically removed and the valve re-repaired. At 12 years old, SaO2 82%, mean PA pressure 12 mm Hg, PA (Nakata) index 111, and pulmonary resistance calculated as 1.4 units m2. The extracardiac Fontan was established by placing an 18 mm ePTFE tube in an off-pump fashion under simple cross-clamping of the inferior caval vein [4], without fenestration. Postoperative trans-pulmonary gradient was 10 mm Hg. The patient was extubated 6 h later. Pleural drainage was needed for 34 days. No arrhythmia occurred throughout. Catheterization 1 year later showed; confluent pulmonary arteries (Fig. 2), PA pressure 11 mm Hg, SaO2 96%, ventricular end diastolic volume 92% of the anticipated norm, and cardiac index 2.1 L/min/m2. He is currently doing well on warfarin, currently 15 years old.
to state, at least at present, that the Fontan circulation is achievable unless MAPCAs are in their extreme forms, such as those requiring extensive reconstruction using prosthetic materials [3], because pulmonary resistance tends to remain higher in those difficult circumstances [6]. In reality, to the best of our knowledge, no other reports are seen in the
2. Discussion Various vascular resistances among the pulmonary segments can persist even after unifocalization, related to thrombo-occlusion of the high resistance areas [3]. Unifocalizing PA at an earlier stage with sacrificing high resistance segments is one strategy for biventricular repair [5], but no consensus in terms of eligibility for the Fontan circulation. Together with the reported case [2], it is sensible
Fig. 2. Inferior caval venous angiography 1 year after the Fontan completion. All the major pulmonary arterial branches were well opacified, although the left artery was underdeveloped.
T. Tsunekawa et al. / International Journal of Cardiology 120 (2007) e3–e5
literature for the Fontan completion following unifocalization of MAPCAs regardless of atrial situs. We previously hypothesized [6]; the Fontan circulation might become possible where MAPCAs were unifocalized efficiently in time. In this patient, the pulmonary circulation was modified relatively early; the left MAPCA was adjusted in infancy, while the right-sided procedure before 2 years old. Nevertheless, the definitive unifocalization was possibly late for the left lung to develop the optimal vasculature. It posed one morbid event, and, consequently, the left PA was slender compared with the right side. Some deleterious circumstances such as protein loosing enteropathy could follow in the longer terms. Progressive atrioventricular regurgitation was another complicating factor. Structural fragility is known to inhere in the common inlet valve. One strategy in hand was for replacing the valve using a mechanical prosthesis and continuously maintaining the pulmonary circulation through the shunts. Persistent cyanosis and lasting anticoagulation should have been the case in such a circumstance. We took the alternative choice to offload the heart. The Fontan pathway proved feasible with improved pulmonary resistance; persevering home oxygen inhalation therapy appeared effective. The course thus far and the prognosis in the future in this patient should be suggestive in the field of adult congenital heart disease, since this is one extreme form of complex
e5
cardiac malformation reaching the Fontan circulation just at the vestibule of adolescence. Acknowledgement We greatly thank Dr Fukiko Ichida and Dr Arata Murakami for their collaboration. References [1] Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Staged unifocalization and anatomic repair in a patient with right isomerism. Ann Thorac Surg 2001;71:2039–41. [2] Miyaji K, Nagata N, Matsui H, Miyamoto T, Kitahori K. Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003;126: 1648–50. [3] Yagihara T, Yamamoto F, Nishigaki K, et al. Unifocalization for pulmonary artesia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1996;112:392–402. [4] Shiraishi S, Uemura H, Kagisaki K, Koh M, Yagihara T, Kitamura S. The off-pump Fontan procedure by simply cross-clamping the inferior caval vein. Ann Thorac Surg 2005;79:2083–8. [5] Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995;109: 832–45. [6] Uemura H, Yagihara T, Ishizaka T, Yamashita K. Pulmonary circulation after biventricular repair in patients with major systemic-to-pulmonary collateral arteries. Eur J Cardiothorac Surg 1997;12:581–6.
Letter to the editor
A case with heterotaxy reaching the Fontan procedure after unifocalizations Tomohiro Tsunekawa a , Toru Ishizaka a , Hideki Uemura b , Koji Kagisaki a , Ikuo Hagino a , Toshikatsu Yagihara a,⁎ b
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan Department of Cardio-thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
Received 1 January 2007; accepted 17 February 2007 Available online 11 June 2007
Abstract One patient with major aorto-pulmonary collateral arteries and heterotaxy underwent staged unifocalizations, and eventually the staged Fontan completion. Subsequent to the bidirectional Glenn procedure, the left pulmonary artery was once thrombozed, but successfully treated. The progressively regurgitant common atrioventricular valve needed repair twice during the course. Pulmonary arterial pressure was 11 mm Hg 1 year after the Fontan procedure. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Heterotaxy; Fontan; Univentricular heart; Pulmonary arteries
In the rare setting of major aortopulmonary collateral arteries (MAPCAs) with heterotaxy, a few successful surgical cases have been reported for anatomic repair [1], or the Fontan procedure [2]. We herein add another patient. 1. Clinical summary A young man with right isomerism of the atrial appendage had common inlet right ventricle (apex pointing right). The aorta (left arch) arose from the dominant ventricle with pulmonary atresia. Two MAPCAs maintained the pulmonary circulation. The pulmonary artery (PA) had recognizable intra-pericardial portions. The atria possessed virtually no septum, receiving all the pulmonary veins unobstructed and the bilateral superior and the left-sided inferior caval veins. The first operation took place at another hospital when 6 months old. Through a left thoracotomy, a 4 mm expanded polytetrafluoroethylene (ePTFE) tube was interposed be-
⁎ Corresponding author. National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. Tel.: +81 6 6833 5012; fax: +81 6 6872 7486. E-mail address: [email protected] (T. Yagihara). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.02.052
tween the subclavian artery and the central PA. Intending to protect the pulmonary vasculature from the progressive hypertensive change, the left MAPCA origin was banded. These markedly improved segmental imbalance in lung perfusion (Fig. 1B and C). He was referred to us for contemplating strategies. At 18 months old, arterial oxygen saturation (SaO2) was 70%. Angiography illustrated a favorable anatomy for unifocalization (Fig. 1A). Pulmonary flow was augmented through a systemic-to-pulmonary shunt of a 5 mm ePTFE tube (right thoracotomy). The right MAPCA was directly implanted to the intra-pulmonary artery [3]. One year later, SaO2 was 79%, mean PA pressure as high as 51 mm Hg, and Qp/Qs value 1.0. At 7 years old, SaO2 75%, mean PA pressure 17 mm Hg, and Qp/Qs 0.7. The left shunt was renewed using a 5 mm ePTFE tube (repeated left thoracotomy). Concomitantly, the MAPCA previously banded was implanted to the central PA. Home oxygen inhalation therapy was started. During following 3 years, SaO2 and Qp/Qs stayed over 80% and 1.2, respectively. Mean PA pressure decreased from 36 to 13 mm Hg, while moderate atrioventricular regurgitation progressed.
e4
T. Tsunekawa et al. / International Journal of Cardiology 120 (2007) e3–e5
Fig. 1. Angiography after the first palliation (A). The right major aorto-pulmonary collateral artery (MAPCA) exhibited stenosis (wide arrow) at its proximal portion, while the left one had been banded at its origin (dotted arrow). The central pulmonary artery (PA) bridged the upper lobes, and possessed intrapulmonary communications to the segments supplied via collaterals (thin arrow). Scintigraphy before (B) and after (C) the initial palliation. Attenuated perfusion to segments other than the left lower lobe vastly improved.
At 10 years old, the bilateral bidirectional Glenn procedure was carried out maintaining an additional flow to the lungs via a 5 mm ePTFE central shunt. The common atrioventricular valve was annulo-plicated by the so-called ‘figure-of-eight’ method. Fourteen months later, the left PA was found thrombosed. Moderate atrioventricular regurgitation recurred. Thrombi were surgically removed and the valve re-repaired. At 12 years old, SaO2 82%, mean PA pressure 12 mm Hg, PA (Nakata) index 111, and pulmonary resistance calculated as 1.4 units m2. The extracardiac Fontan was established by placing an 18 mm ePTFE tube in an off-pump fashion under simple cross-clamping of the inferior caval vein [4], without fenestration. Postoperative trans-pulmonary gradient was 10 mm Hg. The patient was extubated 6 h later. Pleural drainage was needed for 34 days. No arrhythmia occurred throughout. Catheterization 1 year later showed; confluent pulmonary arteries (Fig. 2), PA pressure 11 mm Hg, SaO2 96%, ventricular end diastolic volume 92% of the anticipated norm, and cardiac index 2.1 L/min/m2. He is currently doing well on warfarin, currently 15 years old.
to state, at least at present, that the Fontan circulation is achievable unless MAPCAs are in their extreme forms, such as those requiring extensive reconstruction using prosthetic materials [3], because pulmonary resistance tends to remain higher in those difficult circumstances [6]. In reality, to the best of our knowledge, no other reports are seen in the
2. Discussion Various vascular resistances among the pulmonary segments can persist even after unifocalization, related to thrombo-occlusion of the high resistance areas [3]. Unifocalizing PA at an earlier stage with sacrificing high resistance segments is one strategy for biventricular repair [5], but no consensus in terms of eligibility for the Fontan circulation. Together with the reported case [2], it is sensible
Fig. 2. Inferior caval venous angiography 1 year after the Fontan completion. All the major pulmonary arterial branches were well opacified, although the left artery was underdeveloped.
T. Tsunekawa et al. / International Journal of Cardiology 120 (2007) e3–e5
literature for the Fontan completion following unifocalization of MAPCAs regardless of atrial situs. We previously hypothesized [6]; the Fontan circulation might become possible where MAPCAs were unifocalized efficiently in time. In this patient, the pulmonary circulation was modified relatively early; the left MAPCA was adjusted in infancy, while the right-sided procedure before 2 years old. Nevertheless, the definitive unifocalization was possibly late for the left lung to develop the optimal vasculature. It posed one morbid event, and, consequently, the left PA was slender compared with the right side. Some deleterious circumstances such as protein loosing enteropathy could follow in the longer terms. Progressive atrioventricular regurgitation was another complicating factor. Structural fragility is known to inhere in the common inlet valve. One strategy in hand was for replacing the valve using a mechanical prosthesis and continuously maintaining the pulmonary circulation through the shunts. Persistent cyanosis and lasting anticoagulation should have been the case in such a circumstance. We took the alternative choice to offload the heart. The Fontan pathway proved feasible with improved pulmonary resistance; persevering home oxygen inhalation therapy appeared effective. The course thus far and the prognosis in the future in this patient should be suggestive in the field of adult congenital heart disease, since this is one extreme form of complex
e5
cardiac malformation reaching the Fontan circulation just at the vestibule of adolescence. Acknowledgement We greatly thank Dr Fukiko Ichida and Dr Arata Murakami for their collaboration. References [1] Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Staged unifocalization and anatomic repair in a patient with right isomerism. Ann Thorac Surg 2001;71:2039–41. [2] Miyaji K, Nagata N, Matsui H, Miyamoto T, Kitahori K. Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003;126: 1648–50. [3] Yagihara T, Yamamoto F, Nishigaki K, et al. Unifocalization for pulmonary artesia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1996;112:392–402. [4] Shiraishi S, Uemura H, Kagisaki K, Koh M, Yagihara T, Kitamura S. The off-pump Fontan procedure by simply cross-clamping the inferior caval vein. Ann Thorac Surg 2005;79:2083–8. [5] Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995;109: 832–45. [6] Uemura H, Yagihara T, Ishizaka T, Yamashita K. Pulmonary circulation after biventricular repair in patients with major systemic-to-pulmonary collateral arteries. Eur J Cardiothorac Surg 1997;12:581–6.