Nonatherosclerotic narrowing of the atrioventricular node artery and sudden death

AbstractObjectives. This study was undertaken to determine whether thickening of the atrioventricular (AV) node artery is a cause of sudden cardiac death.Background. Thickening of the AV node artery has been implicated as a cause of sudden death primarily on the basis of case reports. Few pathologic studies have compared subjects who died of sudden cardiac death with normal control subjects who died traumatically.Methods. The AV node artery in 27 patients with unexplained sudden cardiac death (mean age 24.8 ± 7.4 years) was compared with that in 17 control subjects who died traumatically (mean age 25.6 ± 7.0 years). No anatomic cause of death was found at autopsy in the subjects with sudden death, all of whom died of presumed cardiac arrhythmias. The conduction system of all hearts was studied by semiserial sections and Movat pentachrome stains. At the point of greatest narrowing of the AV node artery, the outer circumference and lumen outline were traced by computerized morphometry, the ratio of enter vessel area to lemea area was calculated and the histopathologic changes were noted.Results. The rank-sum of ratios was significantly greater in the sudden death group than in the control group (p = 0.031, Wilcoxon rank-sum/Mann-Whitney statistic). A dysplastic AV node artery with significant acid mucopolysaccharide deposition was seen almost exclusively in the sudden death group (12 of 27 vs. 1 of 17, p = 0.006). In 10 subjects with sudden death a dysplastic AV node artery was narrowed >2 SD over the control value; half of this subgroup died during exercise and one third had a family history of sudden unexplained cardiac death.Conclusions. Dysplasia of the AV node artery may contribute to death in a substantial portion of patients with unexplained sudden death, and such death is often associated with exercise and a family history of unexplained sudden death

TO STUDY THE ROLE OF LIMITED SEQUENCE MAGNETIC RESONANCE IMAGING IN ASSESSMENT OF CHILDREN WITH HYDROCEPHALUS.

Aim: To evaluate the role of limited sequence MRI (LS MRI) in diagnosing obstructive from nonobstructive hydrocephalus for treatment planning correlating with surgical findings and in follow up cases of shunt treated hydrocephalus to predict the candidate requires revision surgery correlating with final treatment. Materials and Methods: A total of 235 cases were included in the study underwent limited sequence MRI, 121 cases were evaluated for diagnosing obstructive from nonobstructive hydrocephalus out of which 106 cases underwent surgery were correlated with surgical findings and 114 were symptomatic follow up cases evaluated for need of revision surgery. Diagnostic measures such as sensitivity, specificity, PPV, NPV and accuracy were calculated. A p value of &lt;0.05 was considered to be statistically significant. Results: Obstruction was seen in 81 out of the 106 cases who underwent surgery. MRI showed obstruction in 72(88.9%) and no obstruction in 9(11.1%) cases. Out of the 25 cases with no obstruction in surgery, MRI correctly excluded obstruction in 20(80%) cases. MRI misdiagnosed obstruction in 5(20%) cases. No statistically significant difference between the limited sequence MRI and surgery (p value of 0.424 Sensitivity 88.89%, Specificity 80% PPV 93.51%, NPV 68.97% and Accuracy 86.79%). Out of the total 114 follow up cases of hydrocephalus,47 underwent surgery and 67 cases were managed conservatively. MRI criteria predicted surgical candidate in 43(91.5%) and no surgery in 4(8.5%) patients. MRI criteria predicted nonsurgical management in 64 (95.5%) out of the 67 cases and the rest of 3 (4.5%) cases MRI over rated need for surgery. (Sensitivity:91.49% Specificity:95.52% PPV: 93.5% NPV 94.1% Accuracy :93.9%.) Conclusion: LS MRI has good accuracy in detecting an obstruction in paediatric hydrocephalus. In predicting revision surgery for follow-up cases of shunt-treated hydrocephalus, LS MRI has good accuracy.</jats:p

Peripartum cardiomyopathy: Clinical, hemodynamic, histologic and prognostic characteristics

Peripartum cardiomyopathy is defined as left ventricular dilation and failure, first developing during the third trimester of pregnancy or in the first 6 months postpartum. In an effort to characterize this syndrome in a middle class population, 14 consecutive patients with peripartum cardiomyopathy underwent a detailed history and physical examination, right heart catheterization, M-mode and two-dimensional echocardiography, radionuclide ventriculography and right ventricular endomyocardial biopsy. These patients were then observed with sequential noninvasive studies to determine prognostic indicators.Eight (57%) of these 14 patients were primiparous and an equal number first presented with heart failure concomitant with or immediately before the onset of labor. When these women were compared with 55 patients with idopathic dilated cardiomyopathy, only mean age at onset of symptoms (28.7 ± 5.7 versus 48.2 ± 13.6 years, p < 0.001) and symptom duration (4.1 ± 7.7 versus 19.0 ± 18.4 months, p < 0.001) differed between the groups. There was no difference in ven- tricular arrhythmia, left ventricular chamber size, ejection fraction or hemodynamics. Myocyte histologic findings were similar; however, myocarditis was identified in 29% of patients with peripartum cardiomyopathy and in only 9% of those with idiopathic dilated cardiomyopathy. In all patients with peripartum cardiomyopathy and myocarditis, the myocardial biopsy was performed within 1 week of onset of symptoms.Seven (50%) of the patients with peripartum cardiomyopathy had dramatic improvement within 6 weeks of follow-up, and 6 (43%) died. Survivors had a higher ejection fraction (22.8 ± 11.7 versus 10.6 ± 1.5%, p < 0.05) and smaller left ventricular cavity size (5.8 ± 1.2 versus 6.9 ± 0.7 cm, p < 0.05). Peripartum cardiomyopathy in a middle class population is hemody-namically indistinguishable from idiopathic dilated cardiomyopathy but is characterized by a high incidence of histologic myocarditis resulting in rapid, spontaneous improvement of congestive heart failure or progressive deterioration resulting in early death