Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants

Schleihauf, Julia; Cleuziou, Julie; Ohain, Jelena Pabst von; Meierhofer, Christian; Stern, Heiko; Shehu, Nerejda; Mkrtchyan, Naira; Kaltenecker, Emanuel; Kühn, Andrea A.; Nagdyman, Nicole; Hager, Alfred; Seidel, Heide; Lange, Rüdiger; Ewert, Peter; Wolf, Cordula M.

doi:10.1093/ejcts/ezx369

Cited by 20 publications

(16 citation statements)

References 26 publications

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Section: Discussionmentioning

confidence: 74%

“…A recent study from Germany reported the clinical outcome of septal myectomy for 12 infant HOCM patients under 1 year of age from 1978 to 2015 including 6 Noonan syndromes [13]. They also confirmed this surgery can be performed safely during infancy with favorable perioperative result and long-term clinical outcome at a mean time period of 8.9 years after surgery [13]. The surgery team from Mayo Clinic also reported the outcome of 7 septal myectomy surgery in patients with Noonan syndrome from 1999 to 2015 [14], which also proved a reasonable and acceptable early outcome for these patients.…”

Section: Discussionmentioning

confidence: 99%

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Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report

Chen

Zhang

2019

BJSTR

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Noonan syndrome is rare disease with autosomal dominant genetic pattern. Hypertrophic obstructive cardiomyopathy (HOCM) is frequently observed in patients with Noonan syndrome and also represents the major determinant of the patient's outcome. The reported experience of septal myectomy for infant Noonan syndrome patient with HOCM is very limited worldwide. In present study, we report a Chinese Noonan syndrome patient with severe neonatal hypertrophic obstructive cardiomyopathy, who underwent septal myectomy procedure at 8 months old. This patient received favorable perioperative result and good clinical outcome after 2 years follow-up. This is the earliest report of septal myectomy surgery for Noonan syndrome patient under 1 year of age, which supports that septal myectomy surgery for infant Noonan syndrome patients with HOCM is scalable with substantial benefit for patients.

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Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 99%

Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report

Chen

Zhang

2019

BJSTR

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“…In a study of 23 pediatric patients with LVOTS undergoing septal myectomy, including 12 patients younger than 1 year of age, Schleihaul et al . [ 1 ] reported that the long-term outcomes were favorable between the infants and older patients. Another study reported that septal myectomy did not increase myocardial extracellular volume fraction and did not affect conduction [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…Left ventricular outflow tract stenosis (LVOTS), a major cause of sudden death in young adults, is usually due to hypertrophic cardiomyopathy in pediatric patients, who can sometimes present with ventricular dysfunction or arrhythmia [ 1 ]. LVOTS often appears with mitral regurgitation (MR) and systolic anterior motion (SAM), with or without extensive hypertrophy of the interventricular septum, and repair of LVOTS remains challenging because of the variety in presentation and patient age.…”

Section: Introductionmentioning

confidence: 99%

Edge-to-edge repair of the systolic anterior motion of mitral valve and cardiac myectomy of the abnormally positioned papillary muscles in an infant with COL4A1 mutation

Miyairi

Takeyoshi

Ishikawa

et al. 2021

Journal of Surgical Case Reports

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Surgical treatment is challenging in pediatric patients with left ventricular outflow tract (LVOT) stenosis (LVOTS). We herein present the case of a 2-year-old male patient with porencephaly who was diagnosed with LVOTS accompanied by moderate mitral valve regurgitation (MR) with systolic anterior motion (SAM). Edge-to-edge mitral valve reconstruction and myectomy of the abnormal cardiac muscle were performed, with an uneventful postoperative course. LVOT myectomy and edge-to-edge mitral valve repair may be considered as a safe and acceptable approach with good clinical outcomes in pediatric patients with LVOTS accompanied by MR with SAM.

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“…This patient cohort was selected for comparison to evaluate similarities and differences between cardiomyopathic changes according to underlying etiology. Data were collected as previously described (44).…”

Section: Patient Populationmentioning

confidence: 99%

Subclinical Cardiac Dysfunction in Childhood Cancer Survivors on 10-Years Follow-Up Correlates With Cumulative Anthracycline Dose and Is Best Detected by Cardiopulmonary Exercise Testing, Circulating Serum Biomarker, Speckle Tracking Echocardiography, and Tissue Doppler Imaging

et al. 2020

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Background: Survivors of childhood cancer are at risk for anthracycline-and/or radiotherapy-induced cardiotoxicity. Aims: The aim of this study was to assess clinical, laboratory, and imaging parameters of subclinical cardiovascular disease in childhood cancer survivors. Methods: Patients underwent cardiopulmonary exercise test (CPET), laboratory testing, transthoracic echocardiography (TTE) with tissue doppler imaging (TDI) and speckle tracking. A subset of patients also underwent cardiovascular magnetic resonance imaging (CMR). Findings were correlated to cumulative anthracycline and exposure to mediastinal irradiation during cancer treatment. In a subgroup analysis, TTE and CMR findings were compared to data from 40 gender-and age-matched patients with childhood onset hypertrophic cardiomyopathy (HCM). Results: Cardiac evaluation was performed in 79 patients (43 males) at 11.2 ± 4.5 years after cancer treatment. Oncologic diagnosis at a median age of 12.0 years was Hodgkin lymphoma in 20, sarcoma in 17, acute leukemia in 24, relapse leukemia in 10, and others in 8 patients. Cumulative anthracycline dose exceeded 300 mg/m 2 in 28 patients. Twenty six patients also received mediastinal irradiation. Decreased peak respiratory oxygen uptake in % predicted on CPET, increased levels of N-terminal pro-brain natriuretic peptide (NTproBNP), increased global longitudinal strain on TTE speckle tracking, and diastolic Wolf et al. Cardiovascular Disease Childhood Cancer Survivors dysfunction on TDI were the most prominent findings on detailed cardiology follow-up. In contrast to HCM patients, childhood cancer survivors did not show left ventricular hypertrophy (LVPWd z-score median 0.9 vs. 2.8, p < 0.001), hyperdynamic systolic function on TTE (Ejection fraction 62 ± 7 vs. 72 ± 12%, p = 0.001), or fibrotic myocardial changes on CMR (Late gadolinium positive 0/13 vs. 13/36, p = 0.001; extracellular volume fraction 22 ± 2 vs. 28 ± 3, p < 0.001) at time of follow-up. There was no correlation between chest radiation exposure and abnormal cardiac findings. Cumulative anthracycline dose was the only significant independent predictor on multivariate analysis for any cardiovascular abnormality on follow-up (p = 0.036). Conclusion: Increasing cumulative anthracycline dose during cancer treatment correlates with subclinical cardiac dysfunction in childhood cancer survivors best detected by elevated cardiac serum biomarkers, decreased exercise capacity on CPET, and abnormalities on echocardiographic speckle tracking and TDI.

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Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants

Abstract: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life.

Cited by 20 publications

References 26 publications

Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report

Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report

Edge-to-edge repair of the systolic anterior motion of mitral valve and cardiac myectomy of the abnormally positioned papillary muscles in an infant with COL4A1 mutation

Subclinical Cardiac Dysfunction in Childhood Cancer Survivors on 10-Years Follow-Up Correlates With Cumulative Anthracycline Dose and Is Best Detected by Cardiopulmonary Exercise Testing, Circulating Serum Biomarker, Speckle Tracking Echocardiography, and Tissue Doppler Imaging

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