‘End-stage’ heart failure therapy: potential lessons from congenital heart disease: from pulmonary artery banding and interatrial communication to parallel circulation

Schranz, Dietmar; Akintuerk, Hakan; Voelkel, Norbert F.

doi:10.1136/heartjnl-2015-309110

Cited by 32 publications

(20 citation statements)

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“…Recent studies reported therapies for end-stage DCM including cardiac resynchronization therapy, restrictive atrial septal defect (ASD) creation, and pulmonary artery banding [4]. Transcatheter ASD creation reduced left atrial pressure and brain natriuretic peptide levels [5].…”

Section: Discussionmentioning

confidence: 99%

Clinical features of Neonatal Cardiomyopathy

Sakurai

Nakano

Masumori

et al. 2019

Asp Biomed Clin Case Rep

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Background: Neonatal cardiomyopathy is a rare disease that ranges from being asymptomatic to abruptly lethal and is not well characterized [1]. We investigated the clinical features of five neonates with cardiomyopathy in our hospital to determine key clinical characteristics. Methods: We retrospectively reviewed the records of five newborns who were diagnosed with cardiomyopathy between January 2000 and December 2018. The primary evaluation included reasons for diagnosis, underlying diseases, therapy, and turning point. Results: Patients with hypertrophic cardiomyopathy (HCM) or left ventricular noncompaction (LVNC) were diagnosed on the basis of cardiac murmur, while the patient with dilated cardiomyopathy (DCM) was diagnosed on the basis of sucking failure. Underlying diseases included Noonan syndrome and LEOPARD syndrome. All patients had received β-blockers, and those with DCM and LVNC were also administered diuretics and angiotensin-converting enzyme inhibitors. The two patients with HCM underwent follow-up as out-patients. One patient with HCM died at 3 years old because of arrhythmia. The patient with DCM died due to heart failure 38 days after birth. The patient with LVNC exhibited severe heart failure after birth, requiring follow-up while considering heart transplantation. Conclusions: Noonan syndrome and LEOPARD syndrome, which is RAS/MAPK-related diseases, should be considered in patients diagnosed HCM. Because heart failure progresses rapidly in patients with neonatal DCM and those with LVNC, planned therapy should include consideration of heart transplantation. Case-1The baby was born by caesarean section at 38 weeks of gestation because of a breech presentation. The infant weighed was 2940 g at birth and her Apgar scores were normal. Her family histories were unremarkable. She was transferred to our hospital because of a cardiac murmur. Echocardiography on admission showed hypertrophy of the interventricular and posterior left ventricular walls (Fig-A). The pressure gradient at the left ventricular outflow tract

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Section: Discussionmentioning

confidence: 99%

Clinical features of Neonatal Cardiomyopathy

Sakurai

Nakano

Masumori

et al. 2019

Asp Biomed Clin Case Rep

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“…On considering pathophysiological-based therapeutic strategies by combining catheter and/or surgical procedures with disease-targeted drug therapies, we might be able to avoid extracorporal life-support with a higher risk/benefit ratio. 7 The fact that an infant with a cytomegalovirus myocarditisassociated heart failure and persistent pulmonary hypertension was kept alive with re-ducting was surprising, though not by the natural setting of a non-restrictive atrial septal defect together with a restrictive ventricular septal defect. We could not determine if the persistence of congenital shunts ameliorated or worsened the viral myocarditis.…”

Section: Discussionmentioning

confidence: 99%

Fulminant cytomegalovirus myocarditis in an infant with concomitant large atrial and ventricular septal defects: medical intervention strategy for functional cardiac regeneration

et al. 2018

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A cytomegalovirus-associated heart failure in a young infant with atrial and ventricular septal defects is reported in this case report. The patient recovered by an anti-congestive and anti-viral therapy with an extra percutaneous transcatheter treatment strategy. In the context of bi-ventricular predominant right heart failure associated with supra-systemic pulmonary hypertension, the already closed arterial duct was re-opened and stented to unload the right ventricle and thereby augment the systemic blood flow. Either the left-to-right shunting atrial septal defect or bi-directional shunting ventricular septal defect was involved in the disease process and was not able to avoid global heart failure. After clinical improvement, the stented duct was shunted left-to-right and was occluded with an ADO-II-AS. During the same procedure the atrial septal defect was closed with an Amplatzer-ASD occluder, while the peri-membranous ventricular septal defect was closed with an ADO-II occluder 2 months later.

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“…Preserved Right Ventricular Function -PRV) u dobnoj skupini prije druge godine života. 68,72 Tran splantaciju srca započeli smo 2011. god. s krivuljom preživljenja od 75%.…”

Section: Raspravaunclassified

Cardiomyopathies in Children

Malčić

Belina

Slišković

et al. 2019

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Introduction: Cardiomyopathies (CM) account for 3-5% of patients in the care of pediatric cardiologists. They are found in all age groups, from fetal to adolescent age, and along with cardiology, teams from several other pediatric subspecialties (neurology, metabolism, genetics) are also included. New findings have led to a high survival rate. Goal: The primary goal is to present CM as an important part in the work of pediatric cardiologist through an elaborate epidemiological study, current classifications, the latest diagnostic methods and treatments, as well as the intertwining with other subspecialties. The secondary goal is to show that CM are no longer “uncommon, insignificant and terminal”, but are common, significant and treatable diseases. Results: From January 1988 to December 2016 (28 years) in the Referral Center for Pediatric Cardiology, Department of Pediatrics, Clinical Hospital Centre, 315 patients were diagnosed with cardiomyopathy,183 males (58.1%) and 132 females (41.9%). In three different periods (10 , 12 and six years) a classification from 1996 was used (10). All three periods have features of a population study, whereas the latter two also have features of an epidemiological study. In all three periods there was a predominance of dilated cardiomyopathies (DCM) (42.5%) , followed by hypertrophic cardiomyopathies (HCM) (37.1%) and restrictive cardiomyopathies (RCM) (6.7%) . Their relative relations were in constant balance. A significant increase of some entity forms, arrhythmogenic right ventricular cardiomyopathies (ARVCM) and non-compaction cardimyopathies (NCCM) has been observed, from 5.8% (1988-1998) to 16.2% (2010-2016). Owing to advances in diagnostic methods, number of unclassified CM has been decreasing significantly. The cause remained unknown in only 24.4% of DCM patients, and in 18.8% of HCM patients. In the last 18 years the mortality rate of 7.4% (14/194) has been recorded, 50% (7/14) due to DCM. That is the result of teamwork, targeted medical therapy, electrotherapy (electrical stimulation – ES, cardiac resynchronisation – CRS, implantable cardioverter defibrilator – ICD) and surgical therapy (Morrow, pulmonary artery banding – PAB), including heart transplantaton since 2011 (8 patients). Conclusion: Cardiomyopathies are after congenital heart defects the most severe diseases under care of pediatric cardiologists. They require fluent teamwork of several expert groups, and mastering of numerous diagnostic and therapeutic methods. Dilated cardiomyopathies are the most common cause of death and the indication for heart transplatation in children.

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‘End-stage’ heart failure therapy: potential lessons from congenital heart disease: from pulmonary artery banding and interatrial communication to parallel circulation

Cited by 32 publications

References 33 publications

Clinical features of Neonatal Cardiomyopathy

Clinical features of Neonatal Cardiomyopathy

Fulminant cytomegalovirus myocarditis in an infant with concomitant large atrial and ventricular septal defects: medical intervention strategy for functional cardiac regeneration

Cardiomyopathies in Children

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