scholarly journals Percutaneous Septal Ablation in Hypertrophic Obstructive Cardiomyopathy: From Experiment to Standard of Care

2014 ◽  
Vol 2014 ◽  
pp. 1-14 ◽  
Author(s):  
Lothar Faber

Hypertrophic cardiomyopathy (HCM) is one of the more common hereditary cardiac conditions. According to presence or absence of outflow obstruction at rest or with provocation, a more common (about 60–70%) obstructive type of the disease (HOCM) has to be distinguished from the less common (30–40%) nonobstructive phenotype (HNCM). Symptoms include exercise limitation due to dyspnea, angina pectoris, palpitations, or dizziness; occasionally syncope or sudden cardiac death occurs. Correct diagnosis and risk stratification with respect to prophylactic ICD implantation are essential in HCM patient management. Drug therapy in symptomatic patients can be characterized as treatment of heart failure with preserved ejection fraction (HFpEF) in HNCM, while symptoms and the obstructive gradient in HOCM can be addressed with beta-blockers, disopyramide, or verapamil. After a short overview on etiology, natural history, and diagnostics in hypertrophic cardiomyopathy, this paper reviews the current treatment options for HOCM with a special focus on percutaneous septal ablation. Literature data and the own series of about 600 cases are discussed, suggesting a largely comparable outcome with respect to procedural mortality, clinical efficacy, and long-term outcome.

2002 ◽  
Vol 39 ◽  
pp. 173 ◽  
Author(s):  
Dirk Welge ◽  
Lothar Faber ◽  
Bernd C. Werlemann ◽  
Leon Krater ◽  
Hubert Seggewiss ◽  
...  

2010 ◽  
Vol 55 (10) ◽  
pp. A37.E356
Author(s):  
Lothar Faber ◽  
Dirk Welge ◽  
Hubert Seggewiss ◽  
Detlef Hering ◽  
Olaf Oldenburg ◽  
...  

2013 ◽  
Vol 61 (10) ◽  
pp. E1228
Author(s):  
Lothar Faber ◽  
Christian Prinz ◽  
Martina Schumann ◽  
Amelie Burghardt ◽  
Hubert Seggewiss ◽  
...  

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Faber ◽  
S Scholtz ◽  
A Batzner ◽  
H Seggewiss

Abstract Introduction and methods Recent registry analyses including the European multicenter registry (Euro-ASA) documented a favourable long-term outcome of septal ablation (ASA/PTSMA) competitive to surgical myectomy. There is no information about the period beyond 10 years. We therefore analyzed the outcome in 120 HOCM patients (pts., mean age @ index intervention: 62±13 years) treated with ASA who survived >10 years. Results Mean CK rise during the index intervention was 537±258 U/l (reference: <80). A DDD-pacemaker (DDD-PM) had to be implanted in 26 pts. (22%) for procedure-related AV conduction problems. A re-intervention for residual or recurrent outflow obstruction (LVOTO) had to be performed in 12 pts. (10%; re-PTSMA: 10 pts.; myectomy: 2 pts). These cases included, at their last follow-up visit 107 pts. (89%) were in functional class I or II. During follow-up (159±33 [range: 122–264] months), 12 pts. (8%) died, of these 6 (4%) from non-cardiac, and 6 (4%) from cardiovascular causes including one sudden death. Furthermore, 4 (3%) ICDs were implanted (1 for secondary, 3 for primary prevention of sudden cardiac death). The most frequent clinical problem was atrial fibrillation which occurred in 27 pts. (23%), and which was refractory to rhythm control attempts in 21 pts. (18%). Conclusions The procedure-related DDD-PM rate in this septal ablation cohort including the early learning curve more than doubled the current one. Nevertheless, during ultra long-term follow up a durable clinical improvement was observed with mortality rates around 1%/year, nearly equivalent to that of 0.8% observed in normal populations. Atrial fibrillation is a frequent problem in this elderly, formerly obstructive HCM population. Funding Acknowledgement Type of funding source: None


2003 ◽  
Vol 41 (6) ◽  
pp. 146
Author(s):  
Maria J. Eriksson ◽  
Anna Woo ◽  
E. Douglas Wigle ◽  
David Focsaneanu ◽  
Paul Rakowski ◽  
...  

2018 ◽  
Vol 6 (1) ◽  
pp. 218
Author(s):  
Nishant Mittal ◽  
Ankit Parakh ◽  
Prashant Jain ◽  
N. K. Mittal

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.


Author(s):  
Denise Schlee ◽  
Till-Martin Theilen ◽  
Henning Fiegel ◽  
Martin Hutter ◽  
Udo Rolle

Summary Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume–outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8 years and 3.2 years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.


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