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PubMed1986

Congenital cardiac defects in calves.

In a 14-year study of calves with cardiac defects, 36 had 78 congenital cardiac defects: ectopia cordia cervicalis (n = 10 defects), common aortic trunk (n = 3 defects), dextraposed aorta (n = 8 defects), duplicated major trunks (n = 1 defect), hypoplastic aorta (n = 2 defects), interventricular septal defect (n = 11), interatrial septal defect (n = 2), left ventricular hypoplasia (n = 10), patent ductus arteriosus (n = 5), patent foramen ovale (n = 5), right ventricular hypoplasia (n = 10), cor triloculare biatriatum (n = 1), endocardial fibroelastosis with calcification (n = 3), and valvular hematomas (n = 7). All septal defects were high in location and ranged from 5 to 35 mm in diameter. One calf with a septal defect also had bilateral microphthalmia.

Animals↗
PubMed1985

Myocyte vacuolization in infarct border zones is reversible.

The nature of the changes occurring in the border zone of myocardial infarcts is uncertain. To study this question, the authors analyzed a number of morphologic features in hearts studied after postmortem arteriography and fixation in distention from 204 patients with single myocardial infarcts autopsied at The Johns Hopkins Hospital. Vacuolization of myocytes was observed in 53 (26%) cases, predominantly in surviving subendocardium and trabecular myocardium within the infarct. Lateral myocardium seldom and subepicardial myocardium almost never showed vacuolar change. Myocyte vacuolization progressively developed and then decreased with time: 1/20 (5%) hearts with infarcts less than 2 days old, 17/48 (35%) infarcts 2-14 days old, 13/27 (48%) infarcts 15-60 days old, 4/12 (33%) infarcts 61-365 days old, and 18/97 (19%) infarcts greater than 365 days old. Reduction in vacuolization with time was not explained by necrosis of vacuolated cells; rather, the myocardium showed normal morphology. Presence of vacuolization in old infarcts was associated with severe multivessel coronary artery disease and endocardial fibroelastosis. The results suggest that infarct border zone myocyte vacuolization may be correctable by reversal of regional ischemia; however, only a trivial amount of myocardium, relative to infarct size, undergoes vacuolar change.

Adult↗
PubMed1978

Endocardial cushion defect and significant hypoplasia of the left ventricle: a distinct clinical and pathological entity.

We have identified 12 patients with endocardial cushion defect and marked under-development of the left ventricle. Most of these patients had significant obstructive anomalies of the aortic arch. Pathologically, the left ventricle was very small, but without endocardial fibroelastosis, significant subaortic narrowing was evident, resulting from maladherent atrioventricular valve tissue to the left ventricular outflow tract, and the left ventricular posterior leaflet component of the atrioventricular valve was both grossly deficient and dysplastic. In all 12 a large ostium primum atrial communication was present, but the ventricular contribution to the defect was small. The clinical presentation of severe, intractable congestive heart failure in these neonatal patients would suggest the diagnosis of typical hypoplastic left heart syndrome. Important departures in some of these patients included a leftward, superior and counterclockwise frontal vector on the electrocardiogram and a widely split second sound. Selective biplane angiocardiography will reveal the underdeveloped left ventricle, with an elongated, fixed, 'gooseneck' deformity of the left ventricular outflow tract, severe 'mitral' regurgitation, and a small ascending aorta. Selective right ventriculography will opacify the very much larger right ventricle and pulmonary artery. Finally, these cases broaden the concept of double inlet right ventricle and exaggerated displacement of the atrioventricular canal towards the bulbus cordis.

Angiocardiography↗
PubMed1977

Myocardial changes after chronic immunization of guinea pigs with diphtheria--tetanus--pertussis (DTP) vaccine.

Fifteen guinea pigs were immunized with diphtheria-tetanus-pertussis [DTP] vaccine once a week throughout the period of 10 months. The control group included 5 guinea pigs. ECG tracings were recorded every second week in both groups of animals. ST-T changes were the most important findings. Sometimes the ECG resembled the picture found in endocardial fibroelastosis in children. ECG abnormalities appeared 3 months after the beginning of the experiment, and were frequently variable. Less pronounced ST-T changes were found also in control animals, but they disappeared after the administration of Inderal. The histological pictures revealed a thickening of the endocardium and degenerative changes of the ganglion cells of the atria; these changes did not always correlate with electrocardiographic tracing.

Animals↗
PubMed1982

[Acute infectious myocarditis (author's transl)].

Among the primary myocardiopathies resulting from a known cause, acute infectious myocarditis plays an important part. This condition, usually interstitial myocarditis of viral etiology, occurs predominantly in the infant under two years of age. They are characterized by a hypotrophic hypokinetic myocardiopathy which is very hard to differentiate from endocardial fibroelastosis. Fibroelastosis is probably only one possible result of the course of certain myocarditis'. However, on the whole, prognosis is good and the children recover. Typhoid fever and diphtheria may also compromise myocardial function.

Acute Disease↗
PubMed1984

Left ventricle-aortic conduits in pediatric patients.

From August, 1974, to January, 1982, left ventricle-aortic porcine valved conduits were inserted in three patients less than 2 years old (Group 1) and in 10 patients between 2 and 14 years of age (Group 2) for relief of severe left ventricular outflow tract obstruction. The distal anastomosis was made to the ascending aorta in seven patients and to the supraceliac abdominal aorta in six patients. In six patients, the conduit was sutured directly to the left ventricle, and in seven a stented right-angle connector was employed. The left ventricle-aortic gradients were relieved in all cases (mean residual gradient = 4.3 mm Hg). All three patients in Group 1 had associated endocardial fibroelastosis and all died. There was one early death in Group 2 (10% mortality). Reoperation was required in seven of nine survivors (78%) 2.7 to 5.2 years postoperatively for conduit valve failure (five patients), progression of mild native aortic valve insufficiency (one patient), or both (one patient). One of the seven required another reoperation for re-replacement of the conduit valve. There was one late death associated with reoperation. At follow-up 3.4 to 7.5 years postoperatively, four patients are in Functional Class I, two are in Class II, and two are convalescing from reoperation. Left ventricle-aortic conduits provide excellent relief of left ventricular outflow tract obstruction. However, the high incidence of late complications suggests better results might be anticipated with aortoventriculoplasty (Konno).

Adolescent↗
PubMed1980

Idiopathic arterial calcification in infancy. Report of a case in a premature fetus.

A case of advanced idiopathic arterial calcification occurred in a macerated male fetus, 29 weeks' gestational age, delivered of a 22-year-old primigravida. The aortic valve, aorta, coronary arteries, ductus arteriosus, and pulmonary, mesenteric, periadrenal, and renal arteries were calcified and were detected on postmortem roentgenogram. Myocardial calcification and endocardial fibroelastosis were also present. The mother's calcium, phosphorus, and alkaline phosphatase levels were normal. Vitamin D intake was not excessive. A maternal febrile illness at 18 to 19 weeks' gestation was the only untoward event during pregnancy.

Aortic Valve↗
PubMed1982

Nonimmunologic hydrops fetalis. A study of ten cases.

Ten cases of hydrops fetalis not associated with serologic incompatibility between mother and infant were studied by autopsy. Classic trisomy 21 was present in two, and in six others the postmortem examination revealed major congenital abnormalities (hydrometrocolpos-polydactyly syndrome, achondrogenesis type 2, congenital adenomatoid malformation of the lung, and aortic valvular dysplasia with left ventricular endocardial fibroelastosis). In two instances, anasarca was associated with a congenital tumor: a sacrococcygeal teratoma and an adrenal neuroblastoma. In six fetuses, prenatal diagnosis of hydrops fetalis or severe congenital malformations was established by ultrasonography.

Abnormalities, Multiple↗
PubMed1981

[Reliability and limitation of various diagnostic methods including nuclear medicine in myocardial disease (author's transl)].

Electrocardiography (ECG), echocardiography, nuclear method, cardiac catheterization, left ventriculography (LVG) and endomyocardial biopsy (biopsy) were performed in 40 cases of cardiomyopathy (CM), 9 of endocardial fibroelastosis (EFE) and 19 of specific heart muscle disease (SHMD), and the usefulness and limitation of each method was comparatively estimated. In CM, various methods including biopsy were performed. The 40 patients were classified into 3 groups, i.e., 1) hypertrophic (17), 2) dilated (20) and 3) non-hypertrophic . non-dilated (3) on the basis of left ventricular ejection fraction (LVEF) and hypertrophy of the ventricular wall assessed by LVG and/or echocardiography. The hypertrophic group was divided into 4 subgroups: 9 septal, 4 apical, 2 posterior and 2 anterior. M-mode scan was useful for detecting hypertrophy at the site of the ventricular septum and posterior wall, but not at the site of the anterior wall and apex. The hypertrophy was detected in 18 out of 20 cases using nuclear method. The posterior wall was hypertrophic but the septum was normal in 2 cases. In 2 of 3 non-hypertrophic . non-dilated cases, the left ventricle was oval in shape by LVG, echocardiography was normal, but significant pathological changes were seen in the biopsy, and there were abnormal ECG findings. There was no correlation between the ECG findings and the site of ventricular hypertrophy. Left ventricular ejection fraction measured by LVG (Kasser) had a closer correlation to LVEF obtained by nuclear method (multigated blood pool scan; r = 0.97) than LVEF by echocardiography (Teichholz; r = 0.79), although LVEF obtained by nuclear method was slightly higher than LVEF obtained by LVG. Myocardial perfusion defect was found in 10 of 20 cases of dilated cardiomyopathy (DCM) and the site of relative hypokinesis coincided with the site of the defect in 6 of 7 cases. A semi-quantitative myocardial perfusion defect index (PDI) and histo pathological contractility failure index (HCFI) obtained by the biopsy were devised. HCFI is the score of myocardial degeneration, fragmentation and fibrosis. The PDI plus HCFI had a close correlation with LVEF (r = -0.898). In 9 cases of DCM, LVEF was more reduced than right ventricular ejection fraction. The perfusion defect was also found in 4 cases of EFE and 4 cases of SHMD, i.e., sarcoidosis, postmyocarditis, Kugelberg-Welander disease and cardiac tumor. We conclude that the nuclear study is useful in assessing the site of the abnormal ventricular thickening, perfusion defect and ventricular function. Echocardiography is most useful in detecting ASH. The biopsy gives the sole diagnostic clue, especially in non-hypertrophic . non-dilated cardiomyopathy. ECG is useful in all cases but correlation with the site of disproportional hypertrophy was not obtained.

Adolescent↗
PubMedDate not supplied

[Myocardial infarct in children. The anatomicoclinical aspects].

Nine cases of myocardial infarction in the newborns (8 cases aged between 7 days and 6 months) and infants (1 case aged 12 years and 6 months) were followed up for 15 years. The etiology was different: mediocalcosis of the coronaries, congenital abnormalities of the heart marked hypertrophy of left ventricular myocardium (endocardial fibroelastosis or nonobstructive hypertrophic cardiomyopathy) severe hypoxia (severe acute bronchopneumopathies), and AAR, cardioarticular form, with multiple attacks in the infant. The ECG aspects were not specific in most children and clinically the prevailing finding was severe decompensated cardiac failure. The main etiologies and the inducing mechanisms of myocardial infarction in children are presented.

Child↗
PubMed1994

Aortic root replacement with pulmonary autograft in children.

Between September 1988 and February 1993, 14 patients whose ages ranged from 3 months to 16 years (mean 11.1 +/- 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1% (95% confidence limits 0% to 21%). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation. Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft. The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiographic follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.

Adolescent↗
PubMed1994

[A case report of successful open valvotomy in neonate with critical aortic stenosis].

Open valvotomy was successfully performed in neonate with critical aortic stenosis using cardiopulmonary bypass. The baby was referred to our hospital at the age of 24 days with very grave state, and needed intensive care including endotracheal intubation and inotropic support. Critical valvular aortic stenosis was confirmed by echocardiography. Poststenotic dilatation and enough size of short axis LV dimension were reported, and aortic annulus was measured 6 mm in diameter. Without catheterization and angiography, open valvotomy was performed with moderate hypothermia and ischemic arrest using single dose of cold cardioplegia at the age of 29 days. Bicuspid aortic valve was thick and dysplastic with thick gelatinous cusp edge, however commissurotomy was applicable in two direction. The diameter of aortic opening was enlarged from 2 mm to 7 mm. Total bypass and aortic cross clamp time were 78 and 28 minutes respectively. The baby recovered uneventfully and there was no evidence of significant AS or aortic regurgitation in echocardiography 7 months after surgery. Sorts of reoperation for restenosis or regurgitation were reported. The results of reoperation for regurgitation were reported to be poor, especially in young infants who should be performed aortic valve replacement. However, residual AS could be manipulated with re-valvotomy, PVB, apico-aortic conduit or AVR. As the choice of first relief of critical AS without other anatomical disadvantages including hypoplastic left ventricle, endocardial fibroelastosis, and mitral stenosis, it would be crucial for late results to prevent progression of aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve↗
PubMed1977

Atrial fibrillation in children.

Atrial fibrillation is rare in children. Previous reports associated it with severe rheumatic heart disease and a poor prognosis. This review is of the unique experience of 35 cases of atrial fibrillation in children in the past 22 years; 23 patients were boys. The age of onset ranged from 1 day to 19 years (average, 8 years). Associated cardiac conditions were severe rheumatic mitral regurgitation (3 cases), cardiomyopathy (5), atrial tumors (2), infective endocarditis (1), paroxysmal atrial tachycardia of infants (4), idiopathic paroxysmal atrial fibrillation (1), Marfan's syndrome with mitral regurgitation (1), endocardial fibroelastosis (1), and structural congenital heart malformations (17). Surgical correction of congenital heart lesions was directly related to the development of atrial fibrillation in 14. Varying arrhythmias of the sick-sinus syndrome were observed in five children. The atrial fibrillation was paroxysmal or transient in 21 patients and persistent in 14. Treatment depended on the underlying condition. Digoxin was used in all cases and cardioversion attempted in ten; no patient was given anticoagulants. Three children had cerebral emboli, with residual defects. Eighteen patients are known to be alive, 13 are dead, and 4 are lost to follow-up. Atrial fibrillation in childhood is an indication for complete investigation of the patient and for the institution of treatment appropriate to the underlying disease.

Adolescent↗
PubMedDate not supplied

Autopsy findings in the Wolcott-Rallison syndrome.

Wolcott-Rallison syndrome is a rare autosomal recessive condition characterized by diabetes mellitus arising in early infancy and multiple epiphyseal dysplasia. To date, nine cases have been described in the world literature. We report an affected girl who died at the age of 4 years and on whom a full autopsy was performed. In addition to neonatal diabetes mellitus and epiphyseal dysplasia, this child had mental retardation and recurrent episodes of self-limiting hepatic failure. Autopsy revealed severe pancreatic hypoplasia and markedly abnormal pancreatic histology, while histology of the bone was consistent with epiphyseal dysplasia. There was laryngeal stenosis and pulmonary hypoplasia. The heart was enlarged with mitral value dysplasia and stenosis, left atrial dilatation, left ventricular hypertrophy, and endocardial fibroelastosis. Examination of the central nervous system showed arrhinencephaly and cerebellar cortical dysplasia. The liver showed minor histological abnormalities but no features were present to account for the recurrent hepatic failure. In addition to Wolcott-Rallison syndrome this child had a deletion at 15q11-12 in 65% of her cells.

Abnormalities, Multiple↗
PubMed1997

Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals.

Isolated noncompaction of the left ventricular myocardium (INVM) is characterized by the presence of numerous prominent trabeculations and deep intertrabecular recesses within the left ventricle, sometimes also affecting the right ventricle and interventricular septum. Familial occurrence of this disorder was described previously. We present a family in which 6 affected individuals demonstrated X-linked recessive inheritance of this trait. Affected relatives presented postnatally with left ventricular failure and arrhythmias, associated with the pathognomonic echocardiographic findings of INVM. The usual findings of Barth syndrome (neutropenia, growth retardation, elevated urinary organic acids, low carnitine levels, and mitochondrial abnormalities) were either absent or found inconsistently. Fetal echocardiograms obtained between 24-30 weeks of gestation in 3 of the affected males showed a dilated left ventricle in one heart, but were not otherwise diagnostic of INVM in any of the cases. Four of the affected individuals died during infancy, one is in cardiac failure at age 8 months, and one is alive following cardiac transplant at age 9 months. The hearts from infants who died or underwent transplantation appeared, on gross examination, to be enlarged, with coarse, deep ventricular trabeculations and prominent endocardial fibroelastosis. Histologically, there were loosely organized fascicles of myocytes in subepicardial and midmyocardial zones of both ventricles, and the myocytes showed thin, often angulated fibers with prominent central clearing and reduced numbers of filaments. Markedly elongated mitochondria were present in some ventricular myocytes from one specimen, but this finding was not reproducible. Genetic linkage analysis has localized INVM to the Xq28 region, where other myopathies with cardiac involvement have been located.

Adolescent↗
PubMed1998

Molecular aspects of myocarditis.

The mechanisms of pathogenesis of myocarditis have remained elusive. Despite the demonstration a decade ago that persistent viral infection of the myocardium occurred in many patients, a clear description of the pathologic progression has not been forthcoming. Over the past year, a number of studies have added to the data defining the crucial roles of cytokine expression in the myocardium and the aberrant induction of apoptosis. Further, a mouse model of myocarditis resulting from the myocardial expression of tumor necrosis factor-alpha has been described. In addition, the identification of the common coxsackievirus B and adenovirus receptor has offered an explanation for the puzzling observation that these highly distinct virus types both cause cardiac disease. Finally, the near-eradication of endocardial fibroelastosis associated with persistent mumps virus infection by vaccination supports the notion that coxsackievirus B and adenovirus vaccines may help reduce the incidence of myocarditis.

Adult↗