A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,... more A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.
A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is ... more A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is herein reported. Successful surgical treatment of this cardiac malformation was accomplished at our institution and consisted of: modified Norwood stage I procedure, with direct anastomosis of the transected main pulmonary artery to the aortic arch, without any prosthetic material interposition and right ventricle
Objective: To assess the risk/bene®t ratio, including cost, associated with routine left atrial c... more Objective: To assess the risk/bene®t ratio, including cost, associated with routine left atrial catheterization for the post-operative management of patients after cardiac surgery. Methods: From November 1991 to June 1998, out of 6187 open heart procedures performed at our institution, 5815 patients (94%) receive a left atrial monitoring line inserted invasively by a unique and reproducible technique. Catheters were removed on the ®rst or second postoperative day before chest tube removal. A subgroup of 385 patients (7%) were switched to a Swan-Ganz catheter postoperatively. Results: A total of 14 patients suffered a complication related to the left monitoring line (0.24%). Bleeding necessitating transfusion occurred in 10 patients (0.17%), seven of whom required surgical reexploration (0.12%). Catheter retention occurred in four cases (0.07%), requiring a mediastinal reexploration in one and a minimally invasive procedures in three, for removal. No other complications related to the presence of a left atrial line emerged in this series. Apart from providing crucial hemodynamic information on a routine basis, in nine selected patients (0.15%) in a low cardiac output state with increased pulmonary vascular resistance and right ventricular failure, the left atrial line was used as a preferential route for catecholamine infusion, with signi®cant hemodynamic improvement. Conclusions: Complications of left atrial monitoring catheters in cardiac surgery do occur but at a very low and acceptable rate. No mortality was correlated to their use in our series. Complication rate can be further lowered by a meticulous management of the device. The wealth of information and therapeutic options offered by this line appears to outweigh the associated risk.
A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is ... more A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is herein reported. Successful surgical treatment of this cardiac malformation was accomplished at our institution and consisted of: modified Norwood stage I procedure, with direct anastomosis of the transected main pulmonary artery to the aortic arch, without any prosthetic material interposition and right ventricle to pulmonary artery polytetrafluoroethylene shunt.
Background. Although deep hypothermic circulatory arrest has been extensively used in neonates fo... more Background. Although deep hypothermic circulatory arrest has been extensively used in neonates for aortic arch surgery, the brain and other organs might be adversely affected by prolonged ischemia and deep hypothermia. Methods. Between December 1997 and January 2005, 70 consecutive neonates underwent Norwood stage I procedure for hypoplastic left heart syndrome (group A, n ؍ 30), or aortic arch repair for interruption or coarctation with arch hypoplasia (group B, n ؍ 40), with antegrade selective cerebral perfusion (ASCP). Mean weights were 3.0 ؎ 0.2 kg and 2.8 ؎ 0.07 kg, and mean ages were 10 ؎ 3.5 days and 14 ؎ 10.6 days in groups A and B, respectively. Only 2 patients were older than 30 days. Core body temperature was lowered to 25°C, and mean pump flow during ASCP was initiated at 10 to 20 mL/(kg • min) and adjusted to guarantee a radial/temporal artery pressure of 30 to 40 mm Hg and venous oxygen saturation of more than 70%. Hematocrit was maintained at 30%. Results. Early mortality was 17% (group A, 23%; group B, 12.5%; p ؍ 0.19). Six late deaths occurred (3 in each group), and at 36 months, Kaplan-Meier overall survival was 64% ؎ 9.2% in group A and 85% ؎ 5.7% in group B. One patient had postoperative seizures. Age, weight, sex, prematurity, group A, and ASCP duration did not influence early mortality. Conclusions. Antegrade selective cerebral perfusion is a safe and effective procedure and might improve outcome of neonatal aortic arch surgery, minimizing neurologic impact without the need for deep hypothermia.
DOI:€10.1016/j.jtcvs.2006.02.041 J Thorac Cardiovasc Surg 2006;132:66-71 Cochrane, Yves d'Ude... more DOI:€10.1016/j.jtcvs.2006.02.041 J Thorac Cardiovasc Surg 2006;132:66-71 Cochrane, Yves d'Udekem d'Acoz and Christian P. Brizard David Michael McMullan, Guido Oppido, Nelson Alphonso, Andrew Donaldartery reconstruction Evaluation of downsized homograft conduits for right ventricle–to–pulmonaryhttp://jtcs.ctsnetjournals.org/cgi/content/full/132/1/66 located on the World Wide Web at: The online version of this article, along with updated information and services, is
OBJECTIVES Italy has been one of the countries most severely affected by the coronavirus disease ... more OBJECTIVES Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patien...
American journal of medical genetics. Part A, 2018
Myhre syndrome is a rare autosomal dominant disorder caused by a narrow spectrum of missense muta... more Myhre syndrome is a rare autosomal dominant disorder caused by a narrow spectrum of missense mutations in the SMAD4 gene. Typical features of this disorder are distinctive facial appearance, deafness, intellectual disability, cardiovascular abnormalities, short stature, short hands and feet, compact build, joint stiffness, and skeletal anomalies. The clinical features generally appear during childhood and become more evident in older patients. Therefore, the diagnosis of this syndrome in the first years of life is challenging. We report a 2-year-old girl diagnosed with Myhre syndrome by whole exome sequencing (WES) that revealed the recurrent p.Ile500Val mutation in the SMAD4 gene. Our patient presented with growth deficiency, dysmorphic features, tetralogy of Fallot, and corectopia (also known as ectopia pupillae). The girl we described is the youngest patient with Myhre syndrome. Moreover, corectopia and tetralogy of Fallot have not been previously reported in this disorder.
Experimental studies showed that extracardiac total cavopulmonary connection provides superior he... more Experimental studies showed that extracardiac total cavopulmonary connection provides superior hemodynamics than atriopulmonary Fontan. We prospectively assessed the impact of conversion of atriopulmonary Fontan to extracardiac total cavopulmonary connection on exercise capacity and cardiac function in 6 consecutive patients. Six months after conversion to extracardiac total cavopulmonary connection, we observed an increase in peak oxygen uptake in all patients (p=0.01;+17%). This improvement was associated to an increase of peak O(2) pulse (p=0.01;+16%), but no change in peak heart rate, arterial oxygen saturation at peak exercise, and pulmonary function. Ventricular ejection fraction did not change significantly after surgery. Conversion was associated with an improvement in heart failure symptoms as assessed by the New York Heart Association classification. Patients who had undergone additional anti-arrhythmia surgery for atrial fibrillation had no recurrence of arrhythmia at follow-up. Data indicate that conversion to extracardiac total cavopulmonary connection is associated with an improvement of cardiopulmonary function and heart failure symptoms. Improved exercise capacity is due to an increase in O(2) pulse and may reflect an improved cardiac stroke volume after the operation.
... un'insidia diagnostica Carlo Pace Napoleone, Guido Oppido, Emanuela Angeli, Gaetano Garg... more ... un'insidia diagnostica Carlo Pace Napoleone, Guido Oppido, Emanuela Angeli, Gaetano Gargiulo ... 3. Holzer R, Johnson R, Ciotti G, Pozzi M, Kitchiner D. Review of an institutional experience of coronary arterial fistulas in childhood set in context of review of the literature. ...
Accurate quantification of aortic dilatation is critical in children with syndromes associated wi... more Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values wer...
A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,... more A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.
An 11-month-old infant, moderately cyanotic, with diagnosis of tetralogy of Fallot underwent corr... more An 11-month-old infant, moderately cyanotic, with diagnosis of tetralogy of Fallot underwent corrective surgery. At echocardiographic evaluation, a subatretic right ventricular outflow tract without aortopulmonary collateral arteries, confluent pulmonary arteries and normal coronary pattern were evidenced. During operation, an undiagnosed large coronary-to-pulmonary artery fistula was disclosed. Because of the large variety of aortopulmonary collateral arteries that can be associated with this pathology, further imaging study is mandatory when oxygen saturation does not match the right ventricular outflow tract obstruction severity.
Multimedia Manual of Cardio-Thoracic Surgery, 2012
Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation... more Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation in some congenital heart disease. In the beginning, when the use of cardiopulmonary bypass was affected by many deleterious effects, this technique played a fundamental role in the treatment of patients with congenital heart defects and an intracardiac left-to-right shunt. The use of PAB has decreased during the last two decades, due to the increasing popularity of early complete intracardiac repair, which results have shown to be superior to staged repair, even in low body weight patients. Moreover, several authors have emphasized the negative effects of PAB such as pulmonary arterial branch distortion, abnormal right ventricular hypertrophy, pulmonary valve insufficiency, sub-aortic obstruction and decreased ventricular compliance in patients with univentricular heart. For all these reasons, this procedure has been placed in the dark corner of surgery, representing, between 2002 and 2005, ~2% of the total amount of cardiac surgery procedures. In a more recent era, PAB has been performed in instances other than classic univentricular heart, as palliation in small infants with cardiac defects with a left-to-right shunt and pulmonary overcirculation, thus gaining some time prior to a planned staged repair. Recently, the role of PAB is becoming more important in selected subsets of congenital cardiac defects: L-transposition of the great arteries, D-transposition of the great arteries, hypoplastic left heart syndrome, moderately hypoplastic left ventricle (congenitally corrected transposition of the great arteries). This renewed interest in the banding procedure is spurring all surgeons and cardiologists to find new solutions for an easier banding procedure while making debanding less traumatic.
A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,... more A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.
A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is ... more A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is herein reported. Successful surgical treatment of this cardiac malformation was accomplished at our institution and consisted of: modified Norwood stage I procedure, with direct anastomosis of the transected main pulmonary artery to the aortic arch, without any prosthetic material interposition and right ventricle
Objective: To assess the risk/bene®t ratio, including cost, associated with routine left atrial c... more Objective: To assess the risk/bene®t ratio, including cost, associated with routine left atrial catheterization for the post-operative management of patients after cardiac surgery. Methods: From November 1991 to June 1998, out of 6187 open heart procedures performed at our institution, 5815 patients (94%) receive a left atrial monitoring line inserted invasively by a unique and reproducible technique. Catheters were removed on the ®rst or second postoperative day before chest tube removal. A subgroup of 385 patients (7%) were switched to a Swan-Ganz catheter postoperatively. Results: A total of 14 patients suffered a complication related to the left monitoring line (0.24%). Bleeding necessitating transfusion occurred in 10 patients (0.17%), seven of whom required surgical reexploration (0.12%). Catheter retention occurred in four cases (0.07%), requiring a mediastinal reexploration in one and a minimally invasive procedures in three, for removal. No other complications related to the presence of a left atrial line emerged in this series. Apart from providing crucial hemodynamic information on a routine basis, in nine selected patients (0.15%) in a low cardiac output state with increased pulmonary vascular resistance and right ventricular failure, the left atrial line was used as a preferential route for catecholamine infusion, with signi®cant hemodynamic improvement. Conclusions: Complications of left atrial monitoring catheters in cardiac surgery do occur but at a very low and acceptable rate. No mortality was correlated to their use in our series. Complication rate can be further lowered by a meticulous management of the device. The wealth of information and therapeutic options offered by this line appears to outweigh the associated risk.
A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is ... more A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is herein reported. Successful surgical treatment of this cardiac malformation was accomplished at our institution and consisted of: modified Norwood stage I procedure, with direct anastomosis of the transected main pulmonary artery to the aortic arch, without any prosthetic material interposition and right ventricle to pulmonary artery polytetrafluoroethylene shunt.
Background. Although deep hypothermic circulatory arrest has been extensively used in neonates fo... more Background. Although deep hypothermic circulatory arrest has been extensively used in neonates for aortic arch surgery, the brain and other organs might be adversely affected by prolonged ischemia and deep hypothermia. Methods. Between December 1997 and January 2005, 70 consecutive neonates underwent Norwood stage I procedure for hypoplastic left heart syndrome (group A, n ؍ 30), or aortic arch repair for interruption or coarctation with arch hypoplasia (group B, n ؍ 40), with antegrade selective cerebral perfusion (ASCP). Mean weights were 3.0 ؎ 0.2 kg and 2.8 ؎ 0.07 kg, and mean ages were 10 ؎ 3.5 days and 14 ؎ 10.6 days in groups A and B, respectively. Only 2 patients were older than 30 days. Core body temperature was lowered to 25°C, and mean pump flow during ASCP was initiated at 10 to 20 mL/(kg • min) and adjusted to guarantee a radial/temporal artery pressure of 30 to 40 mm Hg and venous oxygen saturation of more than 70%. Hematocrit was maintained at 30%. Results. Early mortality was 17% (group A, 23%; group B, 12.5%; p ؍ 0.19). Six late deaths occurred (3 in each group), and at 36 months, Kaplan-Meier overall survival was 64% ؎ 9.2% in group A and 85% ؎ 5.7% in group B. One patient had postoperative seizures. Age, weight, sex, prematurity, group A, and ASCP duration did not influence early mortality. Conclusions. Antegrade selective cerebral perfusion is a safe and effective procedure and might improve outcome of neonatal aortic arch surgery, minimizing neurologic impact without the need for deep hypothermia.
DOI:€10.1016/j.jtcvs.2006.02.041 J Thorac Cardiovasc Surg 2006;132:66-71 Cochrane, Yves d'Ude... more DOI:€10.1016/j.jtcvs.2006.02.041 J Thorac Cardiovasc Surg 2006;132:66-71 Cochrane, Yves d'Udekem d'Acoz and Christian P. Brizard David Michael McMullan, Guido Oppido, Nelson Alphonso, Andrew Donaldartery reconstruction Evaluation of downsized homograft conduits for right ventricle–to–pulmonaryhttp://jtcs.ctsnetjournals.org/cgi/content/full/132/1/66 located on the World Wide Web at: The online version of this article, along with updated information and services, is
OBJECTIVES Italy has been one of the countries most severely affected by the coronavirus disease ... more OBJECTIVES Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patien...
American journal of medical genetics. Part A, 2018
Myhre syndrome is a rare autosomal dominant disorder caused by a narrow spectrum of missense muta... more Myhre syndrome is a rare autosomal dominant disorder caused by a narrow spectrum of missense mutations in the SMAD4 gene. Typical features of this disorder are distinctive facial appearance, deafness, intellectual disability, cardiovascular abnormalities, short stature, short hands and feet, compact build, joint stiffness, and skeletal anomalies. The clinical features generally appear during childhood and become more evident in older patients. Therefore, the diagnosis of this syndrome in the first years of life is challenging. We report a 2-year-old girl diagnosed with Myhre syndrome by whole exome sequencing (WES) that revealed the recurrent p.Ile500Val mutation in the SMAD4 gene. Our patient presented with growth deficiency, dysmorphic features, tetralogy of Fallot, and corectopia (also known as ectopia pupillae). The girl we described is the youngest patient with Myhre syndrome. Moreover, corectopia and tetralogy of Fallot have not been previously reported in this disorder.
Experimental studies showed that extracardiac total cavopulmonary connection provides superior he... more Experimental studies showed that extracardiac total cavopulmonary connection provides superior hemodynamics than atriopulmonary Fontan. We prospectively assessed the impact of conversion of atriopulmonary Fontan to extracardiac total cavopulmonary connection on exercise capacity and cardiac function in 6 consecutive patients. Six months after conversion to extracardiac total cavopulmonary connection, we observed an increase in peak oxygen uptake in all patients (p=0.01;+17%). This improvement was associated to an increase of peak O(2) pulse (p=0.01;+16%), but no change in peak heart rate, arterial oxygen saturation at peak exercise, and pulmonary function. Ventricular ejection fraction did not change significantly after surgery. Conversion was associated with an improvement in heart failure symptoms as assessed by the New York Heart Association classification. Patients who had undergone additional anti-arrhythmia surgery for atrial fibrillation had no recurrence of arrhythmia at follow-up. Data indicate that conversion to extracardiac total cavopulmonary connection is associated with an improvement of cardiopulmonary function and heart failure symptoms. Improved exercise capacity is due to an increase in O(2) pulse and may reflect an improved cardiac stroke volume after the operation.
... un'insidia diagnostica Carlo Pace Napoleone, Guido Oppido, Emanuela Angeli, Gaetano Garg... more ... un'insidia diagnostica Carlo Pace Napoleone, Guido Oppido, Emanuela Angeli, Gaetano Gargiulo ... 3. Holzer R, Johnson R, Ciotti G, Pozzi M, Kitchiner D. Review of an institutional experience of coronary arterial fistulas in childhood set in context of review of the literature. ...
Accurate quantification of aortic dilatation is critical in children with syndromes associated wi... more Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values wer...
A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,... more A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.
An 11-month-old infant, moderately cyanotic, with diagnosis of tetralogy of Fallot underwent corr... more An 11-month-old infant, moderately cyanotic, with diagnosis of tetralogy of Fallot underwent corrective surgery. At echocardiographic evaluation, a subatretic right ventricular outflow tract without aortopulmonary collateral arteries, confluent pulmonary arteries and normal coronary pattern were evidenced. During operation, an undiagnosed large coronary-to-pulmonary artery fistula was disclosed. Because of the large variety of aortopulmonary collateral arteries that can be associated with this pathology, further imaging study is mandatory when oxygen saturation does not match the right ventricular outflow tract obstruction severity.
Multimedia Manual of Cardio-Thoracic Surgery, 2012
Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation... more Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation in some congenital heart disease. In the beginning, when the use of cardiopulmonary bypass was affected by many deleterious effects, this technique played a fundamental role in the treatment of patients with congenital heart defects and an intracardiac left-to-right shunt. The use of PAB has decreased during the last two decades, due to the increasing popularity of early complete intracardiac repair, which results have shown to be superior to staged repair, even in low body weight patients. Moreover, several authors have emphasized the negative effects of PAB such as pulmonary arterial branch distortion, abnormal right ventricular hypertrophy, pulmonary valve insufficiency, sub-aortic obstruction and decreased ventricular compliance in patients with univentricular heart. For all these reasons, this procedure has been placed in the dark corner of surgery, representing, between 2002 and 2005, ~2% of the total amount of cardiac surgery procedures. In a more recent era, PAB has been performed in instances other than classic univentricular heart, as palliation in small infants with cardiac defects with a left-to-right shunt and pulmonary overcirculation, thus gaining some time prior to a planned staged repair. Recently, the role of PAB is becoming more important in selected subsets of congenital cardiac defects: L-transposition of the great arteries, D-transposition of the great arteries, hypoplastic left heart syndrome, moderately hypoplastic left ventricle (congenitally corrected transposition of the great arteries). This renewed interest in the banding procedure is spurring all surgeons and cardiologists to find new solutions for an easier banding procedure while making debanding less traumatic.
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