Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective
Background: Septal reduction therapy via septal myectomy or a modified Konno procedure is the mainstay of therapy for drug-refractory obstructive hypertrophic cardiomyopathy (HCM), although outcomes data on septal myectomy in pediatric patients are limited. We evaluated long-term outcomes following...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-12-01
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| Series: | JTCVS Open |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666273623003054 |
| _version_ | 1797385023102386176 |
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| author | Stephanie N. Nguyen, MD Megan M. Chung, BA Alice V. Vinogradsky, BA Marc E. Richmond, MD, MS Warren A. Zuckerman, MD Andrew B. Goldstone, MD, PhD Emile A. Bacha, MD |
| author_facet | Stephanie N. Nguyen, MD Megan M. Chung, BA Alice V. Vinogradsky, BA Marc E. Richmond, MD, MS Warren A. Zuckerman, MD Andrew B. Goldstone, MD, PhD Emile A. Bacha, MD |
| author_sort | Stephanie N. Nguyen, MD |
| collection | DOAJ |
| description | Background: Septal reduction therapy via septal myectomy or a modified Konno procedure is the mainstay of therapy for drug-refractory obstructive hypertrophic cardiomyopathy (HCM), although outcomes data on septal myectomy in pediatric patients are limited. We evaluated long-term outcomes following surgery for obstructive HCM in a pediatric cohort. Methods: We retrospectively reviewed patients age ≤18 years with obstructive HCM who underwent a left and/or right ventricular septal myectomy at our institution between 1992 and 2022. Primary endpoints were transplantation-free survival, freedom from HCM-related death, and cumulative probability of HCM-related reintervention. We further evaluated outcomes in patients with and without Noonan syndrome or other RASopathies. Results: Thirty-seven patients (median age, 7.4 years; interquartile range [IQR], 3.4-12.9 years) underwent transaortic septal myectomy. A combined modified Konno procedure was performed in 5 patients (13.9%). Sixteen patients (43.2%) had a RASopathy. A concomitant right ventricular outflow tract resection was performed in 9 patients (24.3%). There was 1 (2.7%) in-hospital death and 4 late deaths at a median follow-up of 10.5 years (IQR, 0.1-29.3). Twenty-year transplant-free survival and freedom from HCM-related death were 80.6% (95% confidence interval [CI], 64.2%-100%) and 87.1% (95% CI, 71.8%-100%), respectively. The 20-year cumulative probability of HCM-related reintervention was 34.2% (95% CI, 12.8%-57.1%). Seven patients required a septal reintervention. There was no difference in any primary endpoints between patients with and without a RASopathy. Conclusions: Surgery for obstructive HCM, including septal myectomy with and without a modified Konno procedure, may be performed with low morbidity and good long-term outcomes in pediatric patients. Recurrent outflow tract obstruction is not uncommon. |
| first_indexed | 2024-03-08T21:48:07Z |
| format | Article |
| id | doaj.art-79def80dcc344506b20f69d0a8d8de34 |
| institution | Directory Open Access Journal |
| issn | 2666-2736 |
| language | English |
| last_indexed | 2024-03-08T21:48:07Z |
| publishDate | 2023-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JTCVS Open |
| spelling | doaj.art-79def80dcc344506b20f69d0a8d8de342023-12-20T07:38:20ZengElsevierJTCVS Open2666-27362023-12-0116726738Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspectiveStephanie N. Nguyen, MD0Megan M. Chung, BA1Alice V. Vinogradsky, BA2Marc E. Richmond, MD, MS3Warren A. Zuckerman, MD4Andrew B. Goldstone, MD, PhD5Emile A. Bacha, MD6Section of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYDivision of Pediatric Cardiology, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYDivision of Pediatric Cardiology, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NY; Address for reprints: Emile A. Bacha, MD, Section of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, 3959 Broadway, Babies North, Suite 274, New York, NY 10032.Background: Septal reduction therapy via septal myectomy or a modified Konno procedure is the mainstay of therapy for drug-refractory obstructive hypertrophic cardiomyopathy (HCM), although outcomes data on septal myectomy in pediatric patients are limited. We evaluated long-term outcomes following surgery for obstructive HCM in a pediatric cohort. Methods: We retrospectively reviewed patients age ≤18 years with obstructive HCM who underwent a left and/or right ventricular septal myectomy at our institution between 1992 and 2022. Primary endpoints were transplantation-free survival, freedom from HCM-related death, and cumulative probability of HCM-related reintervention. We further evaluated outcomes in patients with and without Noonan syndrome or other RASopathies. Results: Thirty-seven patients (median age, 7.4 years; interquartile range [IQR], 3.4-12.9 years) underwent transaortic septal myectomy. A combined modified Konno procedure was performed in 5 patients (13.9%). Sixteen patients (43.2%) had a RASopathy. A concomitant right ventricular outflow tract resection was performed in 9 patients (24.3%). There was 1 (2.7%) in-hospital death and 4 late deaths at a median follow-up of 10.5 years (IQR, 0.1-29.3). Twenty-year transplant-free survival and freedom from HCM-related death were 80.6% (95% confidence interval [CI], 64.2%-100%) and 87.1% (95% CI, 71.8%-100%), respectively. The 20-year cumulative probability of HCM-related reintervention was 34.2% (95% CI, 12.8%-57.1%). Seven patients required a septal reintervention. There was no difference in any primary endpoints between patients with and without a RASopathy. Conclusions: Surgery for obstructive HCM, including septal myectomy with and without a modified Konno procedure, may be performed with low morbidity and good long-term outcomes in pediatric patients. Recurrent outflow tract obstruction is not uncommon.http://www.sciencedirect.com/science/article/pii/S2666273623003054hypertrophic cardiomyopathyRASopathyNoonan syndrometransaortic septal myectomy |
| spellingShingle | Stephanie N. Nguyen, MD Megan M. Chung, BA Alice V. Vinogradsky, BA Marc E. Richmond, MD, MS Warren A. Zuckerman, MD Andrew B. Goldstone, MD, PhD Emile A. Bacha, MD Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective JTCVS Open hypertrophic cardiomyopathy RASopathy Noonan syndrome transaortic septal myectomy |
| title | Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective |
| title_full | Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective |
| title_fullStr | Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective |
| title_full_unstemmed | Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective |
| title_short | Long-term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortCentral MessagePerspective |
| title_sort | long term outcomes of surgery for obstructive hypertrophic cardiomyopathy in a pediatric cohortcentral messageperspective |
| topic | hypertrophic cardiomyopathy RASopathy Noonan syndrome transaortic septal myectomy |
| url | http://www.sciencedirect.com/science/article/pii/S2666273623003054 |
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