An 8-year-old spayed Golden Retriever was examined because of recent onset of congestive heart failure complicated by ventricular arrhythmias. Echocardiography revealed a thickened, hypokinetic region of the left ventricular free wall. Fluoroscopically guided transarterial endomyocardial biopsy of the hypokinetic region of the left ventricle revealed infiltration of the endocradium by neoplastic cells consistent with hemangiosarcoma. The dog's clinical condition deteriorated 7 weeks later, and necropsy confirmed the diagnosis and primary site of origin of the tumor. This report describes the clinical use of an endomyocardial biopsy technique to diagnose an infiltrating myocardial tumor that was associated with signs mimicking canine dilated cardiomyopathy.
This study was performed to evaluate the effects of radiofrequency catheter ablation in the coronary sinus as a potential means of eliminating conduction over left‐sided accessory pathways in humans. Radiofrequency current at a frequency of 500 kHz was delivered by electrode catheter to two sites in the coronary sinus of each often dogs. Left coronary arteriography with venous phase visualization of the coronary sinus was performed before, immediately after, and 2 weeks following ablation. Unipolar electrograms from the ablating electrode were recorded before and immediately after ablation. Coronary arteriography revealed no evidence of damage to the adjacent left circumflex coronary artery or its branches as a result of ablation. Contrast visualization of the coronary sinus showed persistent contrast staining following ablation at two of the ablated sites. Angiographically apparent stenosis of the coronary sinus was seen both acutely and chronically in two cases. Unipolar electrogram recordings from the ablating electrode showed an increase in atrial repolarization voltage (atrial current of injury) of 1.53 ± 1.03 mV (P = 0.00004), and an increase in ventricular repolarization voltage of 0.73 ± 0.84 mV (P = 0.005). There was a 23% decrease in amplitude of atrial electrograms (P = 0.006) and a 7% decrease in amplitude of ventricular electrograms (P = 0.02) recorded from the ablating electrode following ablation. Lesions could be identified grossly and microscopically at 16 of the 20 ablated sites. Perforation of the coronary sinus did not occur. Microscopical observation showed normal healing with granulation tissue and fat necrosis extending outward from the coronary sinus involving the atrial epicardium in 13 lesions, the ventricular epicardium in 5 lesions, and the adventitia of the left circumflex coronary artery in 5 lesions. No medial or intimal involvement of the coronary artery was seen. The coronary sinus itself showed luminal organized thrombus in 4 lesions, with near occlusion of the lumen in 1 case. Radiofrequency ablation in the coronary sinus thus results in lesions of a size and extent that would be expected to successfully ablate some left‐sided accessory pathways if delivered in humans. Monitoring of the unipolar electrogram provides insight into the extent of injury during ablation. In some cases, thrombosis of the coronary sinus occurs, the long‐term effects of which are not known.
Introduction: Large scale forward genetic screens with ethyl-nitroso-urea (ENU) mutagenesis can provide a non-gene biased approach to elucidate the genetic etiology of congenital heart disease (CHD). We are using noninvasive fetal echocardiography to elucidate the genetic etiology of CHD with scanning of 100,000 mouse fetuses to achieve 5 fold genome coverage. Methods: C57BL/6J mice (G0) are ENU mutagenized and the resulting G1 males and their G2 daughters are backcrossed to generate G3 fetuses for ultrasound scanning. Primary screening used the clinical Acuson Sequoia ultrasound system with a 15 MHz transducer. Fetuses with CHD were further scanned at 40 MHz with the Vevo 2100 ultra-high frequency biomicroscope (UBM). CHD diagnoses were susbsequently confirmed by necropsy, microCT, and/or histopathology. Results: Ultrasound scanning of >20,000 G3 fetuses from >600 G1 pedigrees revealed 4% of fetuses with cardiac defects. A wide spectrum of CHD was observed, including DORV, TGA, PTA, pulmonary/aortic atresia, coarctation, arch anomalies, atrioventricular septal defects (AVSD) and other defects. The most common CHD found was ventricular septal defect. In contrast, hypoplastic left heart syndrome was found in only one mutant line (Figure). We also recovered 10 lines with laterality defects. Heterotaxy mutants exhibited complex CHD, but no heart disease was seen with situs inversus totalis. Overall, the UBM had higher detection sensitivity for cardiac situs (P<0.01), AVSD (P<0.01) and outflow tract malalignment (P<0.01), with 98.3% of the cardiac situs, 91% of the AVSD and 70% of the outflow defects identified by UBM. Conclusions: Our studies showed the integration of conventional ultrasound with the UBM for fetal mouse cardiovascular phenotyping can maximize the recovery of CHD mutants. The wide spectrum of CHD observed in our large scale screen indicates the importance of genetic contribution in all varieties of structural heart disease.
The increasingly widespread use of cocaine in the United States has been accompanied and perhaps exacerbated by the misconception that the drug is not associated with serious medical complications. In particular, the potential for cocaine to precipitate life-threatening cardiac events needs to be reemphasized. We report the clinical and pathological findings in seven people in whom nonintravenous "recreational" use of cocaine was temporally related to acute myocardial infarction, ventricular tachycardia and fibrillation, myocarditis, sudden death, or a combination of these events. We also review data on 19 previously reported cases of cocainerelated cardiovascular disorders. Analysis of all 26 patients indicated the following findings: (1) the cardiac consequences of cocaine abuse are not unique to parenteral use of the drug, since nearly all the patients took the drug intranasally; (2) underlying heart disease is not a prerequisite for cocaine-related cardiac disorders; (3) seizure activity, a well-documented noncardiac complication of cocaine abuse, is neither a prerequisite for, nor an accompanying feature of, cardiac toxicity of cocaine; and (4) the cardiac consequences of cocaine are not limited to massive doses of the drug. Although the pathogenesis of cardiac toxicity of cocaine remains incompletely defined, available circumstantial evidence suggests that cocaine has medical consequences that are equal in importance to its well-documented psychosocial consequences. (N Engl J Med 1986; 315:1438–43.)
Surgicalrior to the development of CT, cardiac and pericardiac masses were evaluated by chest roentgenography, angiocardiography, and echocardiography. In most cases preoperative diagnosis of a specific entity was impossible. Preoperative CT diagnosis of an intrapericardial developmental mass becomes possible because of the presence of fat. fat/fluid level, thick walled cysts, as well as globular calcifications, bones, teeth, water density fluid, and soft tissue. A report of a young woman with a ruptured and infected intrapericardial teratoma and an infant with an intrapericardial bronchial cyst is presented.
The purpose of these studies was to evaluate metabolic behavior in a 4-day reperfusion model in pigs after induction of subendocardial infarction. Two groups of swine [sham and intervention (Int) groups, n = 7) and 10 hearts per group, respectively] were prepared comparably with two surgical procedures separated over 4 days. In the Int group at the time of the first surgery, coronary flow in the left anterior descending (LAD) circulation was partially restricted (by 60%) for 60 min and was then reperfused. LAD myocardium at the time of the second surgery in both groups was extracorporeally perfused aerobically (5.9 +/- 0.2 ml.min-1.g dry wt-1) for 60 min and infused by equilibrium labeling with [U-14C]-palmitate and [5-3H]glucose to estimate fatty acid oxidation and exogenous glucose utilization. During extracorporeal perfusion, regional myocardial shortening and oxygen consumption were comparable between groups despite a marginal impairment in ATP resynthesis by mitochondria (26% decrease, P < 0.071) in Int hearts and a significant decline in mitochondrial respiration (45% decrease in respiratory control rate, P < 0.008; and 41% decrease in state 3 respiration, P < 0.032) as compared with sham hearts. Fatty acid oxidation described by 14CO2 production was 34.00 +/- 4.72 mumol.h-1.g dry wt-1 (averaged from 30-60 min of perfusion) in sham hearts but was decreased (by 48%, P < 0.004) in Int hearts. This reduction in fatty acid utilization may in part be explained by declines in the observed activity of the mitochondrial membrane transporter enzyme, carnitine palmitoyltransferase.(ABSTRACT TRUNCATED AT 250 WORDS)
Congenital heart disease (CHD) has a multifactorial pathogenesis, but a genetic contribution is indicated by heritability studies. To investigate the spectrum of CHD with a genetic pathogenesis, we conducted a forward genetic screen in inbred mice using fetal echocardiography to recover mutants with CHD. Mice are ideally suited for these studies given that they have the same four-chamber cardiac anatomy that is the substrate for CHD.Ethylnitrosourea mutagenized mice were ultrasound-interrogated by fetal echocardiography using a clinical ultrasound system, and fetuses suspected to have cardiac abnormalities were further interrogated with an ultrahigh-frequency ultrasound biomicroscopy. Scanning of 46 270 fetuses revealed 1722 with cardiac anomalies, with 27.9% dying prenatally. Most of the structural heart defects can be diagnosed using ultrasound biomicroscopy but not with the clinical ultrasound system. Confirmation with analysis by necropsy and histopathology showed excellent diagnostic capability of ultrasound biomicroscopy for most CHDs. Ventricular septal defect was the most common CHD observed, whereas outflow tract and atrioventricular septal defects were the most prevalent complex CHD. Cardiac/visceral organ situs defects were observed at surprisingly high incidence. The rarest CHD found was hypoplastic left heart syndrome, a phenotype never seen in mice previously.We developed a high-throughput, 2-tier ultrasound phenotyping strategy for efficient recovery of even rare CHD phenotypes, including the first mouse models of hypoplastic left heart syndrome. Our findings support a genetic pathogenesis for a wide spectrum of CHDs and suggest that the disruption of left-right patterning may play an important role in CHD.
