Complete Heart Block and Fatal Right Ventricular Failure in an Infant

James, Thomas N.; Nichols, Myron M.; Sapire, David W.; DiPatre, Pier Luigi; Lopez, Suzanne M.

doi:10.1161/01.cir.93.8.1588

Cited by 89 publications

(47 citation statements)

References 24 publications

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“…A defective apoptosis could leave in place some accessory communication between the AV pathway and the adjacent ordinary myocardium, and would leave the sinus node in its fetal configuration, eliminating the beneficial evolution into an appropriate mixture and distribution of P cells among transitional cells [10,16]. An exaggerated apoptosis, could provoke blocking disruption of the pathway itself, and can disfigure the sinus node structure or even completely destroy it [1,10,104,105]. Kajstura et al [106], in their study of programmed cell death during cardiac maturation in rats, found that myocyte cell death was absent in the fetal heart while affected the myocardium postnatally, particularly the right ventricle.…”

Section: Apoptosis Of the Cardiac Conduction Systemmentioning

confidence: 99%

“…Sudden unexpected death is one of the clinical characteristics of the long-QT syndrome and has often been documented to be mediated by lethal ventricular arrhythmias. It is logical to anticipate that the normal occurrence of apoptotic cell death during postnatal morphogenesis of the sinus node will periodically distort or suppress normal sinus rhythm [105]. Moreover, in the long-QT syndrome apoptotic destruction involves not only the myocytes of the sinus node but also many local nerves and ganglia [107,108].…”

Section: Long Qt Syndromementioning

confidence: 99%

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Crib death: further support for the concept of fatal cardiac electrical instability as the final common pathway

Ottaviani

Matturri

Rossi

et al. 2003

International Journal of Cardiology

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This work intends to be a review of the current status of knowledge on the cardiac conduction system in the crib death as well as remaining challenges, including reflections upon authors' personal works as well as many studies by others. The cardiac conduction system findings of resorptive degeneration, His bundle dispersion, Mahaim fibers, cartilaginous meta-hyperplasia, persistent fetal dispersion, left sided His bundle, hemorrhage of the atrio-ventricular junction, septation of the bifurcation, atrio-ventricular node dispersion, sinus node hypoplasia, Zahn node, His bundle hypoplasia, atrio-ventricular node and His bundle dualism are hereby discussed by the authors. The cardiac hypotheses postulating that crib death could be due to lethal cardiac arrhythmias or heart block were considered of great interest in the 1970s. After a general abandon of the conduction studies in crib death, the cardiac concept of crib death is gathering a renewed interest, as well as the occurrence of infantile junctional tachycardia. Both the morphological and functional derangement underlying crib death remain poorly understood, assuring that it remains to be a major medical and social problem. Despite the non-specificity of most of the cardiac conduction findings in crib death, we believe that they, in association with altered neurovegetative stimuli, could underlie potentially malignant arrhythmias, providing a morphologic support for the cardiac concept of crib death. D

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Section: Apoptosis Of the Cardiac Conduction Systemmentioning

confidence: 99%

Section: Long Qt Syndromementioning

confidence: 99%

Crib death: further support for the concept of fatal cardiac electrical instability as the final common pathway

Ottaviani

Matturri

Rossi

et al. 2003

International Journal of Cardiology

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“…The pathogenic mechanism may involve the natural process that causes disparity between the thickness of left and right ventricles (James et al 1996). There is a normal reduction in pressure against which the right ventricle must pump as soon as the heart of the new born starts working.…”

Section: Molecular Pathogenesismentioning

confidence: 99%

“…The second mechanism seems to operate in this post-natal right ventricular morphogenesis since a similar process occurs in the post-natal morphogenesis of the brain, where an excess of neurons are removed by apoptosis. De-regulation of this process, which limits apoptosis to the extent that the haemodynamic pressure is maintained, may lead to complete loss of the myocardium (James et al 1996). This may be the mechanism that operates in the case of Uhl's anomaly, where there is failure of cessation of apoptotic signals or anti-apoptotic signals fail to operate in order to rescue the right ventricular myocardium from complete loss.…”

Section: Molecular Pathogenesismentioning

confidence: 99%

Etiopathogenesis of arrhythmogenic right ventricular cardiomyopathy

et al. 2005

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterised by progressive fibrofatty replacement of right ventricular myocardium. Earlier studies described ARVC as non-inflammatory, non-coronary disorder associated with arrhythmias, heart failure and sudden death due to functional exclusion of the right ventricle. Molecular genetic studies have identified nine different loci associated with ARVC; accordingly each locus is implicated for each type of ARVC (ARVC1-ARVC9). So far five genes have been identified as containing pathogenic mutations for ARVC. Though mutations in each of the gene/s indicate disruption of different pathways leading to the condition, the exact pathogenesis of the condition is still obscure. This review tries to understand the pathogenesis of the condition by examining the individual proteins implicated and relate them to the pathways that could play a role in the aetiology of the condition. Cardiac ryanodine receptor (RYR-2), which regulates intra-cellular Ca 2+ concentration by releasing Ca 2+ reserves from the sarcoplasmic reticulum (SR), was the first gene for ARVC. The mutation in this gene is believed to disrupt coupled gating of RYR-2, causing after-depolarisation, leading to arrhythmias followed by structural changes due to altered intra-cellular Ca 2+ levels. Three other genes implicated for ARVC, plakoglobin (Naxos disease), desmoplakin (ARVC8) and plakophilin (ARVC9) have prompted the speculation that ARVC is primarily a disease of desmosomes. But identification of TGFb-3 for ARVC1 and the role of all these three genes (plakoglobin, desmoplakin and plakophilin) in cardiac morphogenesis indicate some kind of signal-transducing pathway disruption in the condition. The finding that ARVC as a milder form of Uhl's anomaly indicates similar ontogeny for the condition. Further, discovery of apoptotic cells in the autopsy of the right ventricular myocardium of ARVC patients does indicate a common pathway for different types of ARVCs, which is more specific for the right ventricular myocardium involving desmosomal plaque proteins, growth factors and Ca 2+ receptors.

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“…Recent reports described a tremendous apoptotic activity during the perinatal period, 3 which may explain the development of dramatic RV failure in the complete forms in neonates, which are the most frequently observed. 4 Histological examination reveals a direct apposition of endocardium to epicardium without a myocardial layer in between, 5,6 leading to a translucent aspect of the …”

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confidence: 99%

Fortuitous Discovery of Partial Uhl Anomaly in a Male Adult

et al. 2010

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W e report the case of a 44-year-old man referred to our institution for suspicion of arrhythmogenic right ventricular (RV) cardiomyopathy. Both familial and personal medical histories were unremarkable, except the notion of a slight unexplored heart murmur during childhood. He decided to undergo a "40s" medical checkup. On presentation to the general practitioner, he had described a slight fatigue for 8 months without concomitant stress. Physical examination revealed no abnormality. ECG showed a type I atrioventricular block and a complete right bundle-branch block ( Figure 1A). Transthoracic echocardiography showed an unusual left ventricle (LV) with a leftward curveted interventricular septum (IVS) and preserved global and segmental LV systolic function, suggestive of high systolic RV pressure or takotsubo cardiomyopathy ( Figure 1B and Movie I in the onlineonly Data Supplement). However further analysis showed normal systolic pulmonary artery pressure and LV filling pressures. The apical 4-chamber view showed an abnormal RV with an aneurysm-like shape of the basal lateral free wall ( Figure 1B and Movie II in the online-only Data Supplement) but with normal kinetics. A first cardiac magnetic resonance image showed biventricular abnormalities, particularly focused on RV wall motion, which justified additional morphological investigations. No abnormal pulmonary venous return was noticed. Invasive coronary angiogram was normal. The patient was later referred to our unit by his attending cardiologist and underwent invasive hemodynamics and biventricular contrast angiography. Cardiac index (pulmonary artery thermodilution) and biventricular volumes were normal. Blood gas content analysis disclosed an intracardiac shunt. RV angiography (Figure 2A and 2B) showed a truncated RV apex and the absence of trabeculations in the anteroapical and inferoapical zones, therefore characterized by a smooth endocardial aspect associated with a major hypokinesia. Other RV segments had normal morphology and kinetics (subtricuspid zone, tricuspid annulus plane systolic excursion, crista supraventricularis, outflow tract, lateral free wall). RV contours showed a marked notch between normal segments and pathological RV apex, also seen retrospectively on magnetic resonance image, contrast echocardiography, and radionuclide angiography ( Figure 1C). During iodine contrast injection, the pigtail catheter induced a transient, clear parietal deformation that retroceded immediately when the catheter was pulled back and fully consistent with a "parchment-like" RV apex (see Figure 2C and 2D and Movie III in the online-only Data Supplement). The LV apex was trabeculated and abnormally attracted by the RV apex, thus deforming the IVS from right to left with a permanent leftward curveted shape but preserved systolic thickening. Fluid-filled pressure measurement at the RV apex showed a clear slowing in both systolic pressure rise and fall with a midsystolic peak of decreased amplitude compared with RV subtricuspid zone. This abnormal pressure s...

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Complete Heart Block and Fatal Right Ventricular Failure in an Infant

Cited by 89 publications

References 24 publications

Crib death: further support for the concept of fatal cardiac electrical instability as the final common pathway

Crib death: further support for the concept of fatal cardiac electrical instability as the final common pathway

Etiopathogenesis of arrhythmogenic right ventricular cardiomyopathy

Fortuitous Discovery of Partial Uhl Anomaly in a Male Adult

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