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PubMedDate not supplied

Evolution of left ventricular diseasein the fetus. Case report.

A fetal case is described that showed a rapid progression from the features of initial left ventricular fibroelastosis at 20 weeks of gestation to a more marked dilation at 22 weeks and finally to a hypoplastic left ventricle with aortic stenosis at 24 weeks of gestation. This case confirms the evolutive character of left ventricular disease during fetal life.

Adult↗
PubMed1982

Sequelae of atriotomy and ventriculotomy on the endocardium, conduction system and coronary arteries.

The endocardium was analyzed in all four chambers of 99 hearts with various types of congenital heart defects in which surgical repair was performed more than 6 weeks before death. The findings were compared with those of normal hearts in similar age groups. In some cases the endocardium was microscopically examined. This study revealed that in many cases all four chambers had fibroelastosis of the endocardium (diffuse regardless of the type of surgery done previously). These data suggest that diffuse fibroelastosis can occur as a result of surgical intervention and may be related to blocked lymphatic drainage. Sudden death in some patients long after surgery for congenital heart disease and the failure of the chambers to regress to normal size in some cases after total surgical repair may be related to fibroelastosis of the chambers. The sinoatrial node may be injured in atriotomy and in the performance of the Mustard procedure. Ventriculotomy may injure the right bundle branch. Ventriculotomy may also injure the coronary supply to the right ventricle or rarely the anterior descending coronary artery.

Adolescent↗
PubMed1978

Aortic atresia with normal left ventricle and intact ventricular septum.

We report a case of aortic atresia with biventricular hypertrophy and death due to cardiac insufficiency (at 5 1/2 days of age). In the necropsy a normally sized left ventricle was observed, with endocardiac fibroelastosis, anomalous mitral arcade, intact interventricular septum and atresia of the aortic valve. The ascending aorta was moderately hypoplasic; there was a foramen ovale and persistent ductus arteriosus. We believe that this is the first case that has been reported of this anomaly occurring with a normal left ventricle and intact ventricular septum.

Aorta↗
PubMed1976

The pathogenesis of the two forms of hypertensive pulmonary vascular disease.

Autopsy observations suggested that lesions of hypertensive pulmonary vascular disease (HPVD) due to elevated venous pressure differ from those with arterial hypertension only. Clinical and pathologic features were reviewed in patients from the Hopkins autopsy files with proved pulmonary hypertension; 50 had venous HPVD due to left-sided congestive heart failure, 50 had arterial HPVD due to congenital malformations, and 15 had idiopathic pulmonary hypertension (IPH). The two forms of HPVD have consistent distinctive histologic changes. In venous HPVD intimal fibroelastosis (IFE) develops in veins and arteries with retention of normal lumen diameters. Intensity of IFE correlates with severity and duration of venous hypertension. Arterial HPVD has IFE in conducting arteries, but the characteristic lesion is cellular intimal proliferation in regulatory arterioles, producing progresssive irreversible lumenal narrowing. Glomoid and angiomatoid lesions appear with prolonged severe arterial hypertension. They do not occur in venous HPVD. Hypertensive arteritis may develop with either form of HPVD. IPH has arterial-type HPVD. IFE of venous HPVD appears to be a response to increased mural tension. Arteriolar intimal cellular proliferations seen in arterial HPVD may be produced by blood flow boundary layer separations. IPH may be explainable as protracted inappropriate pulmonary arteriolar constriction.

Autopsy↗
PubMed2005

Critical aortic stenosis in early infancy: surgical treatment for residual lesions after balloon dilation.

BACKGROUND: The optimal management for critical aortic stenosis in early infancy continues to challenge cardiologists and cardiac surgeons. We present a review of our experience with the surgical treatment of residual aortic valve disease after percutaneous balloon dilation for critical aortic stenosis in early infancy. METHODS: Since 1989, 11 of the 38 patients who survived aortic balloon dilation (28.9%) have undergone surgical treatment for residual aortic valve dysfunction. Median time from aortic balloon dilation to surgical intervention was 7 months (range 1 to 56 months). Residual aortic stenosis was the predominant problem in 8 patients and aortic regurgitation was predominant in 2 patients. RESULTS: Aortic valvuloplasty was possible in 5 children; pulmonary autograft replacement of the aortic valve was performed in 6 children. Two children underwent a Ross-Konno procedure because of annulus hypoplasia and severe left ventricular outflow tract obstruction. Two early deaths occurred after a Ross-Konno procedure, both with findings of severe left ventricular fibroelastosis at the pathologic examination. Median follow-up time was 5 years (range 1 month to 11.9 years). No late deaths occurred. One patient with moderate-severe aortic valve regurgitation after aortic valvuloplasty underwent a successful Ross operation. All 9 patients are asymptomatic and are in good clinical condition. CONCLUSIONS: We are convinced that the best aortic valve in the pediatric age group is the native one, provided it can function acceptably. However, in cases where conservative surgical treatment fails to yield a functional aortic valve, replacement of the valve is indicated, and the best aortic valve substitute in infants is the pulmonary autograft because of its potential for growth.

Aortic Valve Insufficiency↗
PubMed1978

[Apparently primary acute heart failure in infants. Long-term outcome].

Cases of acute cardiac failure which are apparently primary, and occurring in babies or young children have one characteristics clinical picture. There are three etiological agents, which cannot be differentiated clinically: myocarditis, fibroelastosis, and primary non-obstructive cardiomyopathies. When the critical early period, which still carries a high mortality, has been passed, the outlook is relatively favourable. 61 babies or infants of less than 30 months with acute primary cardiac failure were followed up for a period of between 5 and 20 years. 33 of them were considered cured; 22 have minor sequelae (most frequently) left ventricular hypertrophy on X-ray or ECG); 3 have persistent cardiac failure despite treatment; 3 have died after a period of more than 5 years. There is no clinical, X-ray or ECG finding which can predict the longterm outlook in this condition. It is virtually impossible to differentiate between myocarditis and firboelastosis; it seems likely in the majority of cases that we are dealing with different modes of development in the same condition.

Cardiomyopathies↗
PubMed1986

Cardiac transplantation in an 8-month-old female infant with subendocardial fibroelastosis.

On Nov 1, 1984, an orthotopic cardiac transplantation was performed in an 8-month-old female infant with subendocardial fibroelastosis. Because of the advanced state of cardiac failure, the operation was done despite a positive tissue crossmatch for antitoxic donor-specific antibodies. Immunosuppression consisted of high doses of cyclosporine (up to 550 mg/m2 or 30 mg/kg) and steroids. Hypertension and tremor of the extremities, which were attributed to cyclosporine, occurred during the first week but resolved after seven days. No signs of nephrotoxic effects have been noted; however, a severe episode of allograft rejection was detected by endomyocardial biopsy on the seventh postoperative day, and a moderate rejection episode was noted on the 22nd postoperative day. Histologic improvement was seen after treatment with conventional steroid pulses. The patient was discharged on Nov 29, 1984. Complications consisted of four episodes of otitis media caused by Staphylococcus aureus and one rejection episode that was treated on an outpatient basis with an intravenous methylprednisolone sodium succinate pulse. Our experience emphasizes both the feasibility and importance of performing endomyocardial biopsies in infant recipients. Through biopsy, episodes of rejection can be discovered when clinical signs are not yet apparent. Eighteen months after transplantation, the child was developing and growing normally.

Cyclosporins↗
PubMed1985

Endomyocardial biopsy: a review of the literature.

A review of the literature relating to endomyocardial biopsy (EMB) is presented. This is considered important at this time since EMB is being utilized with increasing frequency, particularly for the diagnosis of myocarditis. The development of the technique is briefly outlined. Emphasis is placed on the clinical application of EMB in the various primary cardiomyopathies (dilated, hypertrophic, restrictive, and obliterative), the infiltrative secondary cardiomyopathies (amyloidosis, sarcoidosis, hemochromatosis), myocarditis, as well as such conditions as adriamycin cardiotoxicity, cardiac transplant rejection, and Kawasaki disease. More controversial application of EMB in primary mitral valve prolapse (Barlow's syndrome), idiopathic ventricular arrhythmias, and the elucidation of the enigmatic finding of angina with angiographically normal coronary arteries is detailed. Experience with immunological and biochemical investigation of biopsy material, as well as with virus isolation and drug assays in the myocardium, is alluded to. Complications encountered with this procedure are also discussed, and its future role is contemplated.

Amyloidosis↗
PubMedDate not supplied

Cardiac function assessment in patients with family history of nonhypertrophic cardiomyopathy: a prenatal and postnatal study.

Nonobstructive cardiomyopathies (CMs) may be familial in 20â "55% of cases. Little is known about the role of fetal echocardiography in such cases. We evaluated the cardiac function serially pre- and postnatally in cases with a family history of nonobstructive CM. The fetal and postnatal studies were performed in the echocardiogarphy laboratory at a tertiary institution. Twenty-six cases from 16 families with a family history of CM were studied. Three fetal echocardiograms were performed at or near 18, 25, and 32 weeks of gestation for complete cardiac functional assessment. Postnatally clinical evaluation, electrocardiogram, and an echocardiogram were performed within the first 3 months, with serial reevaluation for those identified with CM. The mean follow-up was 46 +/- 9 months. Abnormal cardiac function was observed in 8 cases (30%). Six had a previously affected sibling, 1 had other family members affected, and 1 had both antecedents. Four had dilated CM diagnosed prenatally of which 1 recovered, 2 died in utero, and 1 died soon after birth. The remaining 4 had normal fetal echoes and were diagnosed with CM in the first 3 months of life. Three had dilated CM with recovery, and 1 had restrictive CM requiring cardiac transplantation. This study demonstrates a high familial recurrence rate of CM. Fetal echo is useful for early diagnosis, although a normal study does not preclude ventricular dysfunction at a later stage, justifying serial prenatal and postnatal evaluation. Early identification may expedite listing for transplantation.

Adult↗
PubMedDate not supplied

The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: angiographic and pathological studies.

Cases of hypoplastic left heart syndrome (HLHS) were studied angiographically in 18 patients and pathologically in 22 patients. They were divided into three subgroups according to the morphological features of the left heart: mitral atresia with aortic atresia (MA/AA), mitral stenosis with aortic atresia (MS/AA) and mitral stenosis with severe aortic stenosis (MS/AS). Patients with MS/AA had a significantly lower right ventricular end-diastolic volume index and more hypokinesis of the right ventricular posterior wall than those with MA/AA. MS/AA not only increased the thickness of the left ventricular posterior wall and interventricular septum but also increased endocardial thickness compared with MA/AA. Myocardial histology revealed more frequent abnormal findings such as myocardial necrosis, calcification and interstitial fibrosis in the mitral stenosis groups (i.e., MS/AA and MS/AS) than in MA/AA. Right ventricular function appeared to be greatly influenced by left heart structure. The presence of larger left ventricular muscle bulk and frequent myocardial damage seen in MS/AA seems disadvantageous to right ventricular end-diastolic volume and right ventricular wall motion.

Analysis of Variance↗
PubMed2004

[Restrictive cardiomyopathy in childhood].

Restrictive cardiomyopathy is a rare heart muscle disease in childhood. By presenting a case report of a 15 year old adolescent, the aim of this article is to describe diagnosis, pathogenesis and therapy of restrictive cardiomyopathy. A review of the literature comprising pediatric studies on restrictive cardiomyopathy serves as a basis to discuss recommendations for therapeutic strategies in pediatric patients with this rare disease.

Adolescent↗