Romanian Registry of Hypertrophic Cardiomyopathy – overview of general characteristics and therapeutic choices at a national level

Ionilă, Paula; Jurcuţ, Ruxandra; Ferariu, Nicoleta; Roşca, Monica; Chivulescu, Monica; Mursă, Adriana; Militaru, Sebastian; Ionescu, A; Caldararu, Cristina; Fruntelată, Ana; Goanţă, Silvia F.; Crişan, Simina; Ionac, Adina; Avram, Anamaria; Frigy, Attila; Sascău, Radu Andy; Arsenescu-Georgescu, Cătălina; Coman, Ioan Mircea; Popescu, Bogdan A.; Ginghină, Carmen; Apetrei, Eduard

doi:10.2478/rjim-2018-0012

Cited by 5 publications

(5 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The current study presents postoperative clinical and echocardiographic data from 83 consecutive patients with HCM and LVOT obstruction who underwent septal myectomy and MV repair in the Cardiomyopathy Center of Bucharest Monza Hospital. Patients’ demographic data were comparable with statistics reported by Romanian Registry of Hypertrophic Cardiomyopathy [ 9 ] or other cohorts of Romanian patients with HCM [ 10 ], with a mean age falling in the sixth decade of life, and with male predominance. Baseline clinical characteristics and echocardiography findings were similar with those from other European centres [ 11 , 12 ].…”

Section: Discussionsupporting

confidence: 74%

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae—A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy

Dorobantu

Iosifescu

Ticulescu

et al. 2022

JCM

View full text Add to dashboard Cite

Background: Anomalies of the mitral apparatus have been shown to contribute to left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (HCM). We report our 5-year single-center experience with a shallow myectomy procedure associated with transaortic mitral valve repair in a cohort of HCM patients. Methods: We studied 83 consecutive patients who underwent surgical treatment of symptomatic left ventricular outflow obstruction. In all study patients, a transaortic shallow septal myectomy was performed. Fibrous or muscular structures connecting the papillary muscles to the septum or free wall were resected, and fibrotic secondary chordae of the anterior mitral valve were cut selectively. Results: We report one death (1.2%) during hospitalization, no iatrogenic ventricular septal defects, and two (2.4%) mitral valve replacements. At discharge, no patients were in New York Heart Association (NYHA) Class III/IV, from 49 (59%) preoperatively. Mean maximal septal thickness decreased from 24 ± 6 to 16 ± 3 mm. Mean outflow gradient decreased from 93 ± 33 to 13 ± 11 mmHg. Grade 3 or 4 mitral regurgitation was noticed in one patient postoperatively, from 32 (39%) before surgery. Conclusions: Shallow septal myectomy associated with secondary mitral valve chordal cutting and papillary muscle mobilization provided excellent results offering adequate treatment of outflow obstruction.

show abstract

Section: Discussionsupporting

confidence: 74%

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae—A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy

Dorobantu

Iosifescu

Ticulescu

et al. 2022

JCM

View full text Add to dashboard Cite

show abstract

“…The overall demographic profile of our study group was previously documented [37]; the average age at enrollment was approximately in the fifth decade of life, with a higher proportion of male participants, analogous to findings obtained from the Romanian Registry of Hypertrophic Cardiomyopathy [38]. As showed in Table 1, there were no significant differences between the AFib+ (N = 25) and AFib− (N = 20) groups in terms of age, gender distribution, history of sudden cardiac death (SCD), presence of implantable cardiac defibrillators (ICD), and LV size and function.…”

Section: Discussionsupporting

confidence: 66%

Impact of rs1805127 and rs55742440 Variants on Atrial Remodeling in Hypertrophic Cardiomyopathy Patients with Atrial Fibrillation: A Romanian Cohort Study

Popa-Fotea,

Oprescu,

Scafa-Udriste

et al. 2023

IJMS

View full text Add to dashboard Cite

Atrial fibrillation (AFib) is characterized by a complex genetic component. We aimed to investigate the association between variations in genes related to cardiac ion handling and AFib in a cohort of Romanian patients with hypertrophic cardiomyopathy (HCM). Forty-five unrelated probands with HCM were genotyped by targeted next-generation sequencing (NGS) for 24 genes associated with cardiac ion homeostasis. Subsequently, the study cohort was divided into two groups based on the presence (AFib+) or absence (AFiB−) of AFib detected during ECG monitoring. We identified two polymorphisms (rs1805127 located in KCNE1 and rs55742440 located in SCN1B) linked to AFib susceptibility. In AFib+, rs1805127 was associated with increased indexed left atrial (LA) maximal volume (LAVmax) (58.42 ± 21 mL/m2 vs. 32.54 ± 6.47 mL/m2, p < 0.001) and impaired LA strain reservoir (LASr) (13.3 ± 7.5% vs. 24.4 ± 6.8%, p < 0.05) compared to those without respective variants. The rs55742440 allele was less frequent in patients with AFib+ (12 out of 25, 48%) compared to those without arrhythmia (15 out of 20, 75%, p = 0.05). Also, AFib+ rs55742440 carriers had significantly lower LAVmax compared to those who were genotype negative. Among patients with HCM and AFib+, the rs1805127 variant was accompanied by pronounced LA remodeling, whereas rs55742440’s presence was related to a milder LA enlargement.

show abstract

“…The overall demographic pro le of our study group was previously documented [16]; the average age at enrollment was approximately in the fth decade of life, with a higher proportion of male participants, analogous to ndings obtained from the Romanian Registry of Hypertrophic Cardiomyopathy [22]. As showed in Table 2, there were no signi cant differences between the AFib+ (N = 25) and AFib-(N = 20) groups in terms of age, gender distribution, history of sudden cardiac death (SCD), presence of implantable cardiac de brillators (ICD), and LV size and function.…”

Section: Discussionsupporting

confidence: 54%

Impact of rs1805127 and rs55742440 Variants on Atrial Remodeling in Hypertrophic Cardiomyopathy Patients with Atrial Fibrillation: A Romanian Cohort Study

Popa-Fotea,

Oprescu,

Scafa-Udriste

et al. 2023

Preprint

View full text Add to dashboard Cite

Background Atrial fibrillation (AFib), the most prevalent cardiac arrhythmia globally, is characterized by a complex genetic component. In hypertrophic cardiomyopathy, the most commonly encountered genetic cardiac disease, atrial fibrillation is frequently reported in about 20 to 25%, its appearance being associated with poor long-term prognosis. The purpose of this study was to investigate the association between variation in genes related to cardiac ion handling and AFib in a cohort of Romanian patients with hypertrophic cardiomyopathy (HCM). Methods Forty-five unrelated probands with HCM were genotyped by targeted next generation sequencing (NGS) for 24 genes associated with cardiac ion channels and ion homeostasis. Subsequently, the study cohort was divided into two groups based on the presence or absence of AFib detected during ECG monitoring. Results We identified two polymorphisms (rs1805127, c.112A > G located in KCNE1 and rs55742440, c.629T > C located in SCN1B) linked to AFib susceptibility. In AFib group, rs1805127 was associated with increased indexed left atrial (LA) maximal volume (LAVmax = 58.42 ± 21 ml/m2 vs LAVmax = 32.54 ± 6.47 ml/m2, p < 0.001) and impaired LA strain reservoir (LASr = 13.3 ± 7.5% vs LASr = 24.4 ± 6.8%, p < 0.05) compared to those without respective variant. The rs55742440 allele was less frequent in patients with AFib (12 out of 25, 48%) compared to those without arrhythmia (15 out of 20, 75%, p = 0.05). Also, AFib + c.629T > C carriers had significantly lower LAVmax compared to those genotype-negative. Conclusion Among patients with HCM and AFib, rs1805127 variant was accompanied by pronounced LA remodeling, whereas rs55742440’s presence was related to a milder LA enlargement.

show abstract

Romanian Registry of Hypertrophic Cardiomyopathy – overview of general characteristics and therapeutic choices at a national level

Abstract: The Romanian registry of HCM illustrates patient characteristics at a national level as well as the gaps in management which need improvement - accessibility to high-end diagnostic tests and invasive methods of treatment.

Cited by 5 publications

References 22 publications

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae—A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae—A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy

Impact of rs1805127 and rs55742440 Variants on Atrial Remodeling in Hypertrophic Cardiomyopathy Patients with Atrial Fibrillation: A Romanian Cohort Study

Impact of rs1805127 and rs55742440 Variants on Atrial Remodeling in Hypertrophic Cardiomyopathy Patients with Atrial Fibrillation: A Romanian Cohort Study

Contact Info

Product

Resources

About