scholarly journals Clinical Features and Echocardiographic Findings in Children with Hypertrophic Cardiomyopathy

2013 ◽  
Vol 59 (6) ◽  
pp. 285-288
Author(s):  
Blesneac Cristina ◽  
Benedek Theodora ◽  
Togănel Rodica ◽  
Benedek I
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Age Of Onset ◽  
Adult Population ◽  
Systemic Disease ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Myectomy ◽  
Sarcomeric Protein ◽  
Asymptomatic Patients ◽  
Left Ventricular Outflow

Abstract Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%). Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

scholarly journals Outcomes following transaortic septal myectomy in hypertrophic cardiomyopathy

2020 ◽  
Vol 7 (4) ◽  
pp. 1270
Author(s):  
Madhusudan Kummari ◽  
Amaresh Rao Malempati ◽  
Surya S. Gopal Palanki ◽  
Kaladhar Bomma
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Mitral Valve ◽  
Cardiac Death ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Myectomy ◽  
Left Ventricular Outflow ◽  
History Of ◽  
The Mean

Background: The objective of the study was to evaluate effect of myectomy and its impact on survival for a period of one year and to identify the co-morbid conditions that would increase the risk of surgery.Methods: The study was conducted on the patients admitted in a single unit of department of cardiothoracic surgery, NIMS, Hyderabad during the period of 2014 to 2018. The study was a retrospective observational study. 21 patients were enrolled in the study after approval from institute ethics committee. All the patients between 7 to 70 years who underwent septal myectomy were included in the study.Results: Out of the 21 patients underwent modified Morrows myectomy 16 (76.2%) were male and 5 (23.8%) were female. The most common presenting symptom was dyspnea (81%), followed chest pain (76%), palpitations (62%) and syncope (38%). 5 (24%) patients had a family history of sudden cardiac death. Preoperative beta blockers were used by 15 (72%) patients. 11 patients had severe mitral regurgitation, out of which 8 patients underwent valve replacement and 3 underwent mitral valve repair. The mean preoperative left ventricular outflow tract obstruction gradient was 86.86 and the mean postoperative gradient was 23.47. 3 patients had implantable cardioverter defibrillator insertions. All patients had symptom relief.Conclusions: Surgical treatment of hypertrophic cardiomyopathy through transaortic septal myectomy is safe and effective method to relieve left ventricular outflow tract obstruction. Mitral valve replacement can be done for cases with structural defect of mitral valve. Early detection and intervention in patients with family history of sudden cardiac death would reduce the risk of death and ensure long term survival.

Secondary chordal resection with septal myectomy for treatment of symptomatic obstructive hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Eilon Ram ◽  
Ehud Schwammenthal ◽  
Rafael Kuperstein ◽  
Tamer Jamal ◽  
Eyal Nahum ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Interquartile Range ◽  
Septal Myectomy ◽  
Ventricular Outflow Tract Obstruction ◽  
Left Ventricular Outflow

Abstract OBJECTIVES Left ventricular outflow tract obstruction causes symptoms of heart failure in most patients with hypertrophic cardiomyopathy. Resection of the secondary mitral valve (MV) chordae has recently been shown to move the MV apparatus posteriorly, thereby eradicating the outflow gradient. The aim of this study was to evaluate whether secondary chordal resection concomitant to septal myectomy improves outcomes. METHODS Between 2005 and 2020, a total of 165 patients underwent septal myectomy without MV repair or replacement in our Medical Center. Secondary MV chordal resection was performed in 60 patients, and their outcomes were compared with those of the remaining 105 patients who did not undergo chordal resection (controls). Mean age was 61 ± 13 and 58 ± 16 years, respectively (P = 0.205). RESULTS There were no in-hospital deaths throughout the entire cohort. Of those patients who underwent secondary chordal resection, New York Heart Association functional class decreased from 3 (interquartile range 2–3) preoperatively to 1 (interquartile range 1–2) postoperatively (P < 0.001), and resting outflow gradient decreased from 91 ± 39 mmHg to 13 ± 8 mmHg (86% change, P < 0.001). Compared with controls, patients who underwent secondary chordal resection had a significant lower resting outflow gradient at follow-up (14 ± 7 mmHg vs 21 ± 15 mmHg, P = 0.002). The rate of moderate or more than moderate mitral regurgitation at 5 years was 2% in the secondary chordal resection group and 5% in the controls (hazard ratio 1.05, confidence interval 0.11–10.32; P = 0.965). CONCLUSIONS In this observational study, we report that secondary chordal resection concomitant to septal myectomy for left ventricular outflow tract obstruction is safe, relieves heart failure symptoms and reduces left ventricular outflow tract gradient in appropriately selected patients.

scholarly journals Septal myectomy complicated by an embolic stroke in a patient with hypertrophic cardiomyopathy: a case report

2021 ◽  
Author(s):  
Muhammad Umer Butt ◽  
Anas Alameh ◽  
Hanad Bashir ◽  
Ahmad Jabri
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
The United States ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Septal Wall ◽  
Septal Myectomy ◽  
Intraventricular Thrombus ◽  
Left Ventricular Outflow

Abstract Background Hypertrophic cardiomyopathy is estimated to affect 1 out of every 500 adults in the United States. One of its main complications is left ventricular outflow obstruction, which may require surgical septal myectomy in severe cases. We report a rare complication of postoperative septal akinesis leading to thrombus formation presenting as an acute ischaemic stroke. Case summary A 48-year-old woman presented with acute stroke two years after surgical septal myectomy for hypertrophic obstructive cardiomyopathy. Diagnostic workup identified an intraventricular thrombus arising in the left ventricular outflow tract. After comprehensive evaluation, it was determined that the thrombus development was a complication of the prior septal myectomy causing focal septal akinesis. Treatment with anticoagulation resulted in improvement of neurological symptoms and resolution of the intraventricular thrombus. Discussion This case illustrates the rarity and unusual presentation of an intracardiac thrombus that arises from septal myectomy site. A thrombus arising in the left ventricular outflow tract, which is characterized by high gradient laminar flow, is highly unusual. This suggests microscopic and macroscopic alteration in the ventricular septal wall structure, as evident by the septal wall akinesis seen on echocardiography. Recognition of this complication is critical to the selection of appropriate anticoagulation as secondary stroke prevention in these patients.

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