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Case Study
Pulmonary valvuloplasty for pulmonary atresia-restrictive ventricular septal defect
Asian Cardiovascular & Thoracic Annals 0(0) 1–5 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314553995 aan.sagepub.com
Liza Jose Reshmi1, Shrinivas Gadhinglajkar1, Thomas Mathew2, Subramanian Venkateshwaran3, Rupa Sreedhar1 and Baiju Dharan2
Abstract Pulmonary atresia with restrictive ventricular septal defect is a rare congenital cardiac anomaly. A Blalock-Taussig shunt and surgical perforation of the atretic pulmonary valve is often performed as the initial palliation. We present our experience of utilizing both transesophageal and epicardial echocardiography during surgical pulmonary valvuloplasty in a 22-day-old neonate with pulmonary atresia with restrictive ventricular septal defect. The atretic pulmonary valve was perforated using a sheath introduced through the pulmonary artery.
Keywords Heart septal defects, ventricular, infant, newborn, pulmonary atresia, pulmonary valve, heart ventricles, tricuspid valve
Introduction Pulmonary atresia (PA) with restrictive ventricular septal defect (RVSD) is a rare congenital cardiac anomaly that differs morphologically from PA with intact ventricular septum (PA-IVS) and PA with unrestrictive ventricular septal defect (PA-VSD).1,2 A BlalockTaussig (BT) shunt and surgical perforation of the atretic pulmonary valve is performed for initial palliation in these patients.3 We present our experience of utilizing transesophageal echocardiography (TEE) and epicardial echocardiography during surgical pulmonary valvuloplasty in a neonate with PA-RVSD.
Case report A 22-day-old boy was brought to our hospital with cyanosis. Preoperative transthoracic echocardiography revealed a valvular PA, a tortuous S-shaped patent ductus arteriosus (PDA), a 4-mm mid-muscular VSD, and adequately sized pulmonary arteries. The right ventricle (RV) was decompressing into the left ventricle through the RVSD at a Doppler pressure gradient of 12 mm Hg. No RV-to-coronary artery connections or
major aortopulmonary collateral arteries (MAPCA) were detected. After commencing artificial ventilation and prostaglandin infusion, the neonate was transferred to the operating room for a BT shunt, PDA interruption, and pulmonary valvuloplasty. Intraoperative TEE revealed a 4-mm mid-muscular RVSD (Figure 1, Video 1) and no infundibular hypertrophy. The tricuspid valve Z-score and tricuspid/mitral valve ratio were 0.99 and 0.796, respectively. The right/left ventricular length and diameter ratios were 0.66 and 0.757, respectively. The pulmonary annulus was 6.5 mm (Z-score 1.04). After creation of a 3-mm right modified BT shunt, pulmonary 1 Department of Anesthesia, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India 2 Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India 3 Department of Cardiology, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India
Corresponding author: Shrinivas Gadhinglajkar, MD, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, Kerala 695011, India. Email: [email protected]
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Figure 1. The ventricular septal defect (white arrow) is seen in (a) midesophageal 4-chamber view and (b) aortic valve long-axis view.
Figure 2. (a) The sheath (arrow) is seen across the atretic pulmonary valve in right ventricular inflow outflow view. (b) Fourchamber view showing the guidewire with a reverberation artifact (arrow) in the right ventricular cavity. (c) Epicardial right ventricular outflow tract view showing the sheath across the pulmonary valve (arrow).
valvuloplasty was performed through the pulmonary artery after inserting an 18G cannula followed by a guidewire and then an 8F sheath across the pulmonary valve under TEE and epicardial echocardiography guidance (Figure 2, Video 2). The PDA was interrupted. After pulmonary valvoplasty, bidirectional flow was detected across the VSD. The neonate was electively ventilated for 72 h in the postoperative period. He was discharged 12 days after the surgery.
Discussion The pathophysiology is dissimilar in PA-VSD, PA-IVS, and PA-RVSD. PA-VSD is characterized by hypoplasia of the pulmonary arteries and MAPCA-dependent pulmonary flow. PA-IVS is associated with suprasystemic RV pressure, variable hypoplasia of the tricuspid valve and RV, and development of RV-coronary artery connections. Differences in the morphological development of the heart and pulmonary circulation in PA-IVS
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and PA-VSD may be attributed to the timing of closure of the ventricular septum in relation to PA development. Kutsche and colleagues4 postulated that in PA-VSD, the PA occurs before closure of the ventricular septum, resulting in development of pulmonary arterial hypoplasia, MAPCA-dependence, and a tortuous PDA. In PA-IVS, the PA occurs later, after completion of cardiac septation, and the pulmonary circulation develops adequately without MAPCAdependence. PA-RVSD is a rare congenital anomaly with features resembling those of PA-IVS, but having the advantage of a well-developed RV (Figure 3). PA occurs much later in the developmental stages and the ventricular septum does not close completely. As a result, the pulmonary arterial tree grows normally and the pulmonary circulation does not need additional blood supply from MAPCA. Shunting across the RVSD limits the increase in RV pressure and permits adequate growth of the tricuspid valve and RV. However, the size of the RVSD may become inadequate to prevent the RV pressure increasing to a suprasystemic level due to spontaneous closure of the muscular VSD in a growing child. Perforation of an atretic pulmonary valve is necessary for growth of the RV and tricuspid valve, which are prerequisites for performing biventricular repair. Pulmonary valvuloplasty to the extent of the annulus may be performed during BT shunt surgery via the
pulmonary artery or RV.5 In addition to a 4-mm VSD, the 8F sheath provided a 2.7-mm pulmonary valve aperture for RV decompression, which was equivalent to achieving complete pulmonary valvuloplasty. Dilating the pulmonary valve to the extent of the annular level may result in severe pulmonary regurgitation in the presence of a functioning BT shunt, and create a short-circuit pathway among the pulmonary artery, RV, aorta, and BT shunt. Hence we limited the dilatation to the size of an 8F sheath. Various echocardiographic parameters are used to assess growth of the RV and tricuspid valve and the suitability for biventricular repair.6 Intraoperative TEE may be useful to assess these parameters which are not clearly ascertained on preoperative echocardiography. A tricuspid valve Z-score < 3 and tricuspid/ mitral ratio
(AAN)
[1–5] [PREPRINTER stage]
Case Study
Pulmonary valvuloplasty for pulmonary atresia-restrictive ventricular septal defect
Asian Cardiovascular & Thoracic Annals 0(0) 1–5 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314553995 aan.sagepub.com
Liza Jose Reshmi1, Shrinivas Gadhinglajkar1, Thomas Mathew2, Subramanian Venkateshwaran3, Rupa Sreedhar1 and Baiju Dharan2
Abstract Pulmonary atresia with restrictive ventricular septal defect is a rare congenital cardiac anomaly. A Blalock-Taussig shunt and surgical perforation of the atretic pulmonary valve is often performed as the initial palliation. We present our experience of utilizing both transesophageal and epicardial echocardiography during surgical pulmonary valvuloplasty in a 22-day-old neonate with pulmonary atresia with restrictive ventricular septal defect. The atretic pulmonary valve was perforated using a sheath introduced through the pulmonary artery.
Keywords Heart septal defects, ventricular, infant, newborn, pulmonary atresia, pulmonary valve, heart ventricles, tricuspid valve
Introduction Pulmonary atresia (PA) with restrictive ventricular septal defect (RVSD) is a rare congenital cardiac anomaly that differs morphologically from PA with intact ventricular septum (PA-IVS) and PA with unrestrictive ventricular septal defect (PA-VSD).1,2 A BlalockTaussig (BT) shunt and surgical perforation of the atretic pulmonary valve is performed for initial palliation in these patients.3 We present our experience of utilizing transesophageal echocardiography (TEE) and epicardial echocardiography during surgical pulmonary valvuloplasty in a neonate with PA-RVSD.
Case report A 22-day-old boy was brought to our hospital with cyanosis. Preoperative transthoracic echocardiography revealed a valvular PA, a tortuous S-shaped patent ductus arteriosus (PDA), a 4-mm mid-muscular VSD, and adequately sized pulmonary arteries. The right ventricle (RV) was decompressing into the left ventricle through the RVSD at a Doppler pressure gradient of 12 mm Hg. No RV-to-coronary artery connections or
major aortopulmonary collateral arteries (MAPCA) were detected. After commencing artificial ventilation and prostaglandin infusion, the neonate was transferred to the operating room for a BT shunt, PDA interruption, and pulmonary valvuloplasty. Intraoperative TEE revealed a 4-mm mid-muscular RVSD (Figure 1, Video 1) and no infundibular hypertrophy. The tricuspid valve Z-score and tricuspid/mitral valve ratio were 0.99 and 0.796, respectively. The right/left ventricular length and diameter ratios were 0.66 and 0.757, respectively. The pulmonary annulus was 6.5 mm (Z-score 1.04). After creation of a 3-mm right modified BT shunt, pulmonary 1 Department of Anesthesia, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India 2 Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India 3 Department of Cardiology, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, India
Corresponding author: Shrinivas Gadhinglajkar, MD, Sree Chitra Tirunal Institute For Medical Sciences and Technology, Trivandrum, Kerala 695011, India. Email: [email protected]
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. The ventricular septal defect (white arrow) is seen in (a) midesophageal 4-chamber view and (b) aortic valve long-axis view.
Figure 2. (a) The sheath (arrow) is seen across the atretic pulmonary valve in right ventricular inflow outflow view. (b) Fourchamber view showing the guidewire with a reverberation artifact (arrow) in the right ventricular cavity. (c) Epicardial right ventricular outflow tract view showing the sheath across the pulmonary valve (arrow).
valvuloplasty was performed through the pulmonary artery after inserting an 18G cannula followed by a guidewire and then an 8F sheath across the pulmonary valve under TEE and epicardial echocardiography guidance (Figure 2, Video 2). The PDA was interrupted. After pulmonary valvoplasty, bidirectional flow was detected across the VSD. The neonate was electively ventilated for 72 h in the postoperative period. He was discharged 12 days after the surgery.
Discussion The pathophysiology is dissimilar in PA-VSD, PA-IVS, and PA-RVSD. PA-VSD is characterized by hypoplasia of the pulmonary arteries and MAPCA-dependent pulmonary flow. PA-IVS is associated with suprasystemic RV pressure, variable hypoplasia of the tricuspid valve and RV, and development of RV-coronary artery connections. Differences in the morphological development of the heart and pulmonary circulation in PA-IVS
Downloaded from aan.sagepub.com at UNIV OF FLORIDA Smathers Libraries on November 10, 2015
XML Template (2014) [3.10.2014–7:23am] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/140217/APPFile/SG-AANJ140217.3d
(AAN)
[1–5] [PREPRINTER stage]
Reshmi et al.
3
and PA-VSD may be attributed to the timing of closure of the ventricular septum in relation to PA development. Kutsche and colleagues4 postulated that in PA-VSD, the PA occurs before closure of the ventricular septum, resulting in development of pulmonary arterial hypoplasia, MAPCA-dependence, and a tortuous PDA. In PA-IVS, the PA occurs later, after completion of cardiac septation, and the pulmonary circulation develops adequately without MAPCAdependence. PA-RVSD is a rare congenital anomaly with features resembling those of PA-IVS, but having the advantage of a well-developed RV (Figure 3). PA occurs much later in the developmental stages and the ventricular septum does not close completely. As a result, the pulmonary arterial tree grows normally and the pulmonary circulation does not need additional blood supply from MAPCA. Shunting across the RVSD limits the increase in RV pressure and permits adequate growth of the tricuspid valve and RV. However, the size of the RVSD may become inadequate to prevent the RV pressure increasing to a suprasystemic level due to spontaneous closure of the muscular VSD in a growing child. Perforation of an atretic pulmonary valve is necessary for growth of the RV and tricuspid valve, which are prerequisites for performing biventricular repair. Pulmonary valvuloplasty to the extent of the annulus may be performed during BT shunt surgery via the
pulmonary artery or RV.5 In addition to a 4-mm VSD, the 8F sheath provided a 2.7-mm pulmonary valve aperture for RV decompression, which was equivalent to achieving complete pulmonary valvuloplasty. Dilating the pulmonary valve to the extent of the annular level may result in severe pulmonary regurgitation in the presence of a functioning BT shunt, and create a short-circuit pathway among the pulmonary artery, RV, aorta, and BT shunt. Hence we limited the dilatation to the size of an 8F sheath. Various echocardiographic parameters are used to assess growth of the RV and tricuspid valve and the suitability for biventricular repair.6 Intraoperative TEE may be useful to assess these parameters which are not clearly ascertained on preoperative echocardiography. A tricuspid valve Z-score < 3 and tricuspid/ mitral ratio
