Sudden cardiac death with apparently normal heart.

BACKGROUND: Mechanisms of sudden cardiac death (SCD) in subjects with apparently normal hearts are poorly understood. In survivors, clinical investigations may not establish normal cardiac structure with certainty. Large autopsy series may provide a unique opportunity to confirm structural normalcy of the heart before reviewing a patient's clinical history. METHODS AND RESULTS: We identified and reexamined structurally normal hearts from a 13-year series of archived hearts of patients who had sudden cardiac death. Subsequently, for each patient with a structurally normal heart, a detailed review of the circumstances of death as well as clinical history was performed. Of 270 archived SCD hearts identified, 190 were male and 80 female (mean age 42 years); 256 (95%) had evidence of structural abnormalities and 14 (5%) were structurally normal. In the group with structurally normal hearts (mean age 35 years), SCD was the first manifestation of disease in 7 (50%) of the 14 cases. In 6 cases, substances were identified in serum at postmortem examination without evidence of drug overdose; 2 of these chemicals have known associations with SCD. On analysis of ECGs, preexcitation was found in 2 cases. Comorbid conditions identified were seizure disorder and obesity (2 cases each). In 6 cases, there were no identifiable conditions associated with SCD. CONCLUSIONS: In 50% of cases of SCD with structurally normal hearts, sudden death was the first manifestation of disease. An approach combining archived heart examinations with detailed review of the clinical history was effective in elucidating potential SCD mechanisms in 57% of cases.

New device for closure of muscular ventricular septal defects in a canine model.

BACKGROUND: Repair of muscular ventricular septal defects (MVSDs) has always been challenging to the surgeon. Long-term morbidity and mortality are significantly increased if the defects are closed via left ventriculotomy or if they are associated with other complex congenital anomalies. The purpose of this study was to close MVSDs with the Amplatz ventricular septal defect device. This device is constructed from 0.004-in nitinol wire mesh filled with polyester fibers. It is retrievable, repositionable, self-centering, and of low profile. METHODS AND RESULTS: MVSDs were created with the help of a sharp punch in 10 dogs. The location of the defects was anterior muscular (n=3), midmuscular (n=3), apical (n=3), and inlet muscular (n=1). The diameter of the defects ranged from 6 to 14 mm. All defects were closed in the catheterization laboratory. The device was placed with the help of transesophageal echocardiography and fluoroscopy. A 7F sheath was used to deploy the device from the right ventricular side in 8 and the left ventricular side in 2 dogs. Placement was successful in all animals. The complete closure rate was 30% (3/10) immediately after placement and 100% at 1-week follow-up. Pathological examination of the heart revealed complete endothelialization of the device in dogs killed after 3 months. CONCLUSIONS: The Amplatz ventricular septal defect device appears highly efficacious in closing MVSDs. The advantages include a small delivery sheath, complete retrievability before release, and the fact that it is self-centering and self-expanding, thereby making it an attractive option in smaller children.

Pulmonary circulation in pulmonary atresia associated with the asplenia cardiac syndrome.

OBJECTIVE: The goal of this study was to determine the patterns of the pulmonary circulation in patients with pulmonary atresia and asplenia. BACKGROUND: The asplenic cardiac syndromes characteristically have complex cardiac anomalies including pulmonary stenosis or atresia. Definition of the pulmonary artery circulation and pulmonary venous connections is needed for consideration of surgical procedures. METHODS: In 35 patients, the sources of pulmonary blood flow, anatomic features of pulmonary arteries and pulmonary venous connections were determined from angiograms or autopsy specimens. RESULTS: The main pulmonary artery was absent or hypoplastic in 91% of patients; most had a ductus arteriosus. The right and left pulmonary arteries were confluent in 90% and usually of normal size (right 71%, left 63%). Total anomalous pulmonary venous connections were present in 38%. CONCLUSIONS: The anatomic features of the pulmonary arteries in pulmonary atresia associated with the asplenic cardiac syndrome are usually favorable for palliative surgical procedures. Total anomalous pulmonary venous connection may exist as a complicating factor.

Incessant ventricular tachycardia in infants: myocardial hamartomas and surgical cure.

Infants with incessant ventricular tachycardia (occurring greater than 10% of the day) have generally been described in pathologic studies. This report describes 21 patients with incessant ventricular tachycardia present greater than 90% of the day and night; the age at diagnosis ranged from birth to 30 months (mean 10.5 months). The most common clinical presentation was cardiac arrest (11 patients, in 5 after digitalis for presumed supraventricular tachycardia); another 6 patients had congestive heart failure and 4 were asymptomatic. Three patients had coexisting Wolff-Parkinson-White syndrome. The rate of incessant ventricular tachycardia ranged from 167 to 440 (mean 260 beats/min) and the QRS duration from 0.06 to 0.11 second. The most common electrocardiographic (ECG) pattern (10 of 21) was right bundle branch block with left axis deviation, but other right and left bundle branch block patterns were observed. Conventional and investigational antiarrhythmic agents (nine patients received amiodarone) failed to eliminate incessant ventricular tachycardia in all. Electrophysiologic studies localized incessant ventricular tachycardia to the left ventricle in 17 (to the apex in 2, the free wall in 9 and the septum in 6) and to the right ventricular septum in 4. No structural abnormalities were found on the echocardiogram or angiocardiogram. All 21 patients had surgery at an age of 3.5 to 31 months (mean 16). In 15 a tumor was found: 13 myocardial hamartomas (9 discrete, 4 diffuse throughout both ventricles) and 2 rhabdomyomas (1 multiple). Myocarditis was found in one patient (the oldest). In four, only myocardial fibrosis was found; results of one biopsy were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Myocardial infarction and percent arteriographic stenosis of culprit lesion: report from the Program on the Surgical Control of the Hyperlipidemias (POSCH).

The objective of this study was to assess the percent stenosis of the culprit lesion responsible for subsequent myocardial infarction in the Program on the Surgical Control of the Hyperlipidemias (POSCH). It is unknown if the susceptible coronary artery culprit lesion responsible for an acute myocardial infarction is relatively large ( > or = 50% arteriographic stenosis) and hemodynamically significant ( > or = 70% stenosis), or small ( < 50%, stenosis) and asymptomatic. Certain necropsy and arteriography studies support the large progenitor lesion concept, and other arteriography studies support the small lesion hypothesis. We analyzed the coronary arteriogram immediately preceding a Q wave (transmural) myocardial infarction for the degree of stenosis of the suspected culprit lesion, which was selected by visual inspection of the coronary circulation supplying the electrocardiogram-defined area of myocardial infarction. There was no perceptible difference with respect to vessel segment distribution of culprit lesions or time to infarction between the 52 control-group patients and the 27 intervention-group patients. For the two groups combined (n=79), the predominantly involved segments were the middle right coronary artery and the proximal left anterior descending coronary artery. The time interval from the preceding coronary arteriogram closest to the index myocardial infarction ranged from 0 days to 10 years; however, 64.6% of the arteriograms were performed 2 years or less prior to the myocardial infarction. Only 5.1% of the patients in both groups combined had a culprit lesion stenosis < 50%, while 88.6% of the patients in both groups combined had a culprit lesion stenosis > or = 70%. The results strongly favor the large lesion hypothesis of causation for myocardial infarction. It is premature, however, to state that the relative size of the culprit lesion has been indisputably determined. The resolution of this problem has exceedingly important practical implications for the management of patients with known atherosclerotic coronary heart disease and for those asymptomatic individuals with silent atherosclerotic coronary heart disease.

Causes of sudden unexpected cardiac death in the first two decades of life.

Sudden, unexpected cardiac death in the age group 1 to 21 years usually is due to myocarditis, hypertrophic cardiomyopathy, aortic valvar stenosis, and coronary arterial abnormalities. The hearts of 70 patients <21 years of age who died suddenly were reviewed. Twenty patients were <1 year of age and 50 were 1 to 21 years old. The cardiac findings were compared with those in 68 age-matched controls with known cardiac disease who did not die suddenly. Significant cardiac abnormalities were present in 13 (65%) of the 20 infants; 10 (50%) had anomalies of the aortic origin of the coronary arteries. Among the 50 older patients, cardiac abnormalities were found in 40 (80%), among whom coronary arterial anomalies existed in 12 (24%). Anomalies of aortic origin more frequently involved the left main than the right coronary artery in both groups.

Congenital stenotic arteriopathy with medial dysplasia.

An unusual, uniformly stenotic arteriopathy involving the aorta and its major branches, the pulmonary trunk, and the left and right pulmonary arteries was found in association with a persistent common atrioventricular canal in an otherwise normal stillborn female infant. The uniform arterial thickening was due to hyperplastic medial elastic laminae, which were in an orderly arrangement in the inner two thirds but dysplastic in the outer third of the media of the arteries.

Left ventricular cellular hypertrophy in pressure- and volume-overload valvular heart disease.

To determine the dependence of myocyte hypertrophy in chronic valvular heart disease on the site and type of lesion, the myocardium was studied from 11 patients with either pressure-overload hypertrophy (PO; four patients with aortic stenosis and two with mixed aortic stenosis/insufficiency) or pure volume-overload hypertrophy (VO; two patients with mitral regurgitation and three with aortic insufficiency). These patients, all without coronary artery disease, died zero to 34 days after valve replacement surgery. Diameters of 25 longitudinally oriented myocytes in the circular midwall myocardium were measured with a calibrated light microscope eyepiece reticle on each of five transmural, transverse, histologic sections from the apical, anterolateral, posterolateral, anteroseptal, and posteroseptal left ventricle. Statistical analysis by modified two-way analysis of variance (ANOVA) demonstrated that mean myocyte size (based on 125 measurements) varied widely among cases but was not statistically different among sites. The myocyte diameter for PO lesions (25.9 +/- 1.1 micron, mean +/- SEM) was significantly greater (P less than 0.05) than that for pure VO lesions (20.4 +/- 0.7 micron), despite equal relative heart weights (measured/predicted from body weight: 2.5 +/- 0.2 [mean +/- SD] versus 2.5 +/- 0.5). This study suggests that 1) cellular hypertrophy in valvular heart disease occurs uniformly throughout the left ventricular myocardium; and 2) mean myocyte diameters are greater in PO than in VO hypertrophy for equivalent cardiac enlargement.

Stenotic semilunar valve in persistent truncus arteriosus.

The clinical, hemodynamic, and pathologic findings in two newborn infants with persistent truncus arteriosus and stenosis of the truncal valve are described. In one case the anatomic features of the basic condition were classic, with a dysplastic semilunar valve which was mainly stenotic and also incompetent, while in the other the truncus arteriosus arose exclusively from the right ventricle and was almost exclusively stenotic. A ventricular septal defect was the only outlet for the left ventricle. In this case, mitral stenosis was also present and associated with a left-to-right shunt at the atrial level.

Cardiac rhabdomyoma simulating mitral atresia.

Clinical, catheterization, and pathologic findings were recorded in a newborn infant with tuberous sclerosis and multiple cardiac rhabdomyomas that produced a clinical picture simulating mitral atresia and the hypoplastic left-heart syndrome. The clinical picture was due to a left atrial tumor that completely obstructed the mitral valvular orifice. Even if the diagnosis of left atrial tumor had been made, successful surgical correction was unlikely because of left ventricular rhabdomyomas, which produced severe subvalvular aortic stenosis and did not appear to be resectable. This case demonstrates the possibility that a hamartoma, such as a rhabdomyoma, occasionally can mimic the hypoplastic left-heart syndrome.

Macroscopic enzyme-mapping verification of large, homogeneous, experimental myocardial infarcts of predictable size and location in dogs.

Difficulty is frequently experienced in producing a large homogeneous myocardial infarct in the dog heart because of the extensive network of coronary anastomoses. This problem may be overcome by combining the ligation of the left anterior descending coronary artery with agar injection into the distal coronary vasculature to obliterate anastomotic channels. All infarcts produced in this manner occupied a constant area in the anterior wall of the left ventricle. From our results in 25 dogs, the individual infarct averaged 12.3 Gm. in weight (range 9.4 to 13.5), representing 25 to 30 per cent of the total left ventricular muscle mass. The homogeneity of the infarct was verified by a simple, macroscopic enzyme-mapping technique based on the inability of the ischemic (dehydrogenase-deficient) myocardium to reduce triphenyl tetrazolium chloride and by detailed histologic studies. Apart from providing ample raw material for comprehensive morphologic, chemical, histochemical, and radioisotopic analyses, a large myocardial infarct also serves as a useful experimental model for various physiological and hemodynamic studies of cardiogenic shock and left ventricular akinesis.

Porcine heterograft valve replacement in carcinoid heart disease.

A 53-year-old man with a pancreatic carcinoid tumor and liver metastases had the carcinoid syndrome with involvement of the tricuspid valve by carcinoid plaque. The mitral valve was involved by unrelated myxomatous degeneration (floppy valve). Each valve was replaced by a Hancock glutaraldehyde-prepared porcine heterograft prosthesis. When the patient died of complications of the tumor 8 months postoperatively, both valves had clinically normal function. Nevertheless, the carcinoid plaque, which was present in all four cardiac chambers and almost completely covered the endocardial surfaces of both atria, extended onto both prostheses. This eventually might have interfered with prosthetic valve function.

Clinically unsuspected disseminated intravascular coagulation (DIC): an autopsy survey.

In a review of 768 consecutive autopsies, 21 (2.7%) clinically unsuspected cases of disseminated intravascular coagulation (DIC) syndrome were diagnosed by histologic examination. DIC was diagnosed by the presence of fibrin thrombi in arterioles, capillaries, venules, and medium-sized veins. Fibrin thrombi were found, in the descending order of frequency, in the brain, heart, lungs, kidneys, adrenals, spleen and liver. Most patients had multiple visceral involvement, with three showing fibrin thrombi in as many as ten organs. The density of fibrin thrombi was greatest in the spleen, followed by kidneys, liver, lungs, adrenals, brain, and heart. A review of clinical data showed that infections were the most common underlying conditions in 13 cases, including nine with positive bacterial cultures from blood or cerebrospinal fluid, or both. The results suggest that, despite increasing clinical recognition of DIC, a great number of patients remain unsuspected of having the DIC syndrome prior to postmortem examination.

Myocardial abscesses.

A review of postmortem materials from two teaching hospitals, accumulated in a 14-year period (1962--1975), disclosed 63 patients with myocardial abscesses among 12,359 autopsies, an incidence of 0.5%. All 63 patients had multi-focal myocardial abscesses; the lesions were grossly discernible in six patients. Coexisting infective endocarditis was present in approximately 20% (12) of the 63 patients with myocardial abscesses. Candida and Staphylococcus aureus were most common organisms responsible for the abscesses. Candida was identified in 23 patients (37%) by histologic examination of the heart sections, and 11 of these also had antemortem blood cultures positive for Candida. Staphylococcus aureus was cultured from the antemortem blood of 22 patients, two of whom also showed candidal organisms in the heart at necropsy. Eighty-one per cent (51) of 63 patients had abscesses in one or more extracardiac organs. It appeared that most of the myocardial abscesses had resulted from disseminated sepsis. Surgical conditions, malignancy and alcoholic hepatic disease were the most frequent primary conditions in patients with myocardial abscesses.

In vivo effect of coronary laser angioplasty on atherosclerotic plaques: histopathologic analysis.

Information from histopathologic examination of coronary arterial atherosclerotic plaques treated with in vivo laser energy is sparse. Directional atherectomy provides biopsies for study of tissue changes (injury) due to coronary arterial debulking devices, including laser. Sixteen patients who presented with acute ischemic coronary syndromes underwent debulking of a total of 17 obstructive intracoronary lesions with pulsed-wave holmium:YAG laser (2.1 microm wavelength). Laser was performed with the "pulse and retreat" technique which incorporates slow catheter advancement (0.5-1 mm/s) with controlled emission of energy. Immediately postlasing, directional atherectomy was utilized to obtain irradiated plaque tissue for pathologic examination. Extent of laser-induced tissue injury to plaques was graded as 0 (no tissue damage), 1 (small foci or charring and vacuoles), 2 (large amount of charring, edge disruption and vacuoles) and 3 (extensive tissue damage). Angiographically and clinically, all 17 lesions were successfully debulked with the laser energy (mean 47+/-25 pulses), with a reduction of target lesion percent diameter stenosis from 92+/-6% to 47+/-25%. Adjunct balloon dilations further reduced the target lesions to a final of 10+/-10% stenosis. The histopathologic examination of the lased specimens demonstrated that 13 lesions (76%) had no evidence of laser-induced injury (Grade 0). Four lesions had low-level injury (Grade 1), and none had evidence of Grade 2 or 3 laser-induced trauma. Therefore, a laser debulking technique, which incorporates slow catheter advancement with controlled emission of pulses, does not cause significant injurious effects to the irradiated plaque.

Cardiac troponin T alterations in myocardium and serum of rats after stressful, prolonged intense exercise.

The goal of this study was to determine whether the stress of forced exercise would result in injury to the myocardium. Male rats with 8% of body weight attached to the tail were forced to swim 3.5 h (3.5S), forced to swim 5 h (5S), or pretrained for 8 days and then forced to swim 5 h (T5S). Rats were killed immediately after they swam (0 h PS) and at 3 h (3 h PS), 24 h (24 h PS), and 48 h after they swam (48 h PS). Tissue homogenates of the left ventricle were analyzed by Western blot analysis for cardiac troponin T (cTnT). Serum cTnT was quantified by immunoassay. Results indicated that, in the 3.5S, 5S, and T5S groups, serum cTnT was significantly (P < 0.01) increased at 0 and 3 h PS. The 5S group demonstrated a greater increase in serum cTnT than the 3.5S group (P < 0.01) and the T5S group (P < 0.01) at 0 h PS. Western blot analysis indicated significant decreases (P < 0. 01) in myocardial cTnT in the 5S group only at 0 h PS (P < 0.01) and 3 h PS (P < 0.05). Histological evidence of localized myocyte damage demonstrated by interstitial inflammatory infiltrates consisting of neutrophils, lymphocytes, and histiocytes, as well as vesicular nuclei-enlarged chromatin patterns, was observed in left ventricle specimens from the 5S group at 24 and 48 h PS. Our findings demonstrate that stressful, forced exercise induces alterations in myocardial cTnT and that training before exercise attenuates the exercise-induced heart damage.

Perventricular [correction of Periventricular] closure of ventricular septal defects without cardiopulmonary bypass.

BACKGROUND: Minimally invasive techniques are currently in use to close atrial and ventricular septal defects (VSD). Cardiopulmonary bypass (CPB) is instituted via the femoral vessels, which may cause injury to these vessels, especially in younger patients. The objectives of this study were to demonstrate the feasibility of perventricular [corrected] closure of muscular VSD (MVSD) and paramembranous VSD (PVSD) without CPB, using the Amplatz VSD device. METHODS: Five Yucatan pigs with naturally occurring PVSD (3- to 7-mm diameter) and 5 dogs with surgically created MVSD (6- to 14-mm diameter) were subjects of this study. The VSDs were closed intraoperatively with a 7-French delivery sheath inserted through the free wall of the right (n = 5) or left ventricle (n = 5), under epicardial echocardiogram guidance. The animals were followed for 3 months. RESULTS: There was no operative mortality. All MVSD closed after placement of the device. Closure rate of PVSD was 4 of 5 after placement and 3 of 5 after 3 months. One pig developed aortic incompetence at the last follow-up. CONCLUSIONS: Perventricular closure of MVSD and PVSD is feasible. Avoidance of CPB can decrease recovery time, its complications, and trauma to the femoral vessels.

Quadricuspid aortic valve and single coronary ostium.

We describe an autopsy patient in whom a rare congenital anomaly of quadricuspid semilunar aortic valve and coronary arteries originating from a single orifice of one aortic sinus occurred. To the best of our knowledge, this combination of cardiac anomalies has not been reported.

Inflammatory pseudotumor (plasma cell granuloma) of the heart. Report of two cases and literature review.

We report pathologic findings in two patients with inflammatory pseudotumor of the heart. The first patient was a 15-year-old-boy who died suddenly and unexpectedly of myocardial ischemia caused by an angiocentric inflammatory pseudotumor affecting all of the major coronary arteries and some of their branches. Inflammatory pseudotumor was also centered around some intrasplenic arteries. The second patient was a 5-month-old girl who had subtotal resection of a mass in the right atrial free wall. Her inflammatory pseudotumor was confined to the myocardium and showed no angiocentricity. The patient is doing well 22 months after surgery. Inflammatory pseudotumor is a benign inflammatory response evoked by an unknown agent(s). In both patients, the inflammatory cells comprised a mixture of B and T lymphocytes. Among B cells, a mixture of kappa and lambda plasma cells was evident. A moderate number of tissue macrophages was also observed. The process is usually self-limited but may cause death if vital structures are involved.

Nonbacterial thrombotic endocarditis (NBTE) and disseminated intravascular coagulation (DIC): autopsy study of 36 patients.

A pathogenetic relationship between nonbacterial thrombotic endocarditis (NBTE) and disseminated intravascular coagulation (DIC) was sought by reviewing autopsies from a recent 12-year period. Of 4,783 autopsies, 36 patients were found to have NBTE. The histopathologic diagnosis of DIC in patients with NBTE was dependent on the observation of thrombi in vasa recta and central glomerular capillaries of the kidneys and in sinusoids, arterioles, capillaries, venules, and medium-sized veins of the major viscera. Morphologic evidence of DIC was found in 18 (50%) of the 36 patients with NBTE. In addition, venous and arterial thromboses were found in 13 patients (36%) and pulmonary thromboembolism in 17 (47%). These findings support the view that NBTE and DIC may be pathogenetically related and result from a hypercoagulable state.