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PubMed1978

Idiopathic familial myocardiopathy in three generations: a clinical and pathologic study.

A peculiar non-hypertrophic myocardiopathy is described which occurred in three and possibly five generations of a single family. Clinical features included systolic murmurs, electrocardiographic abnormalities, and sudden cardiac death with a paucity of symptoms of cardiac dysfunction. Pathological studies in three generations showed a striking similarity of cardiac findings including globular and dilated ventricles, endocardial fibroelastosis, and mitral valve thickening. Myocardium in two showed basophilic degeneration and fibrosis. A retrospective genealogic analysis and a prospective clinical evaluation of living family members suggested an autosomal dominant mode of inheritance with variable penetrance. The cause of this heritable myocardiopathy is presumably a mutant gene; the biochemical defect to which the mutant gene gives rise remains unknown.

Adolescent↗
PubMed2005

Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options.

Apart from complete and incomplete congenital heart block (CHB), new cardiac manifestations related to anti-SSA/Ro antibodies have been reported in children born to mothers bearing these antibodies. These manifestations include transient fetal first-degree heart block, prolongation of corrected QT (QTc) interval, sinus bradycardia, late-onset cardiomyopathy, endocardial fibroelastosis and cardiac malformations. Anti-SSA/Ro antibodies are not considered pathogenic to the adult heart, but a prolongation of the QTc interval has recently been reported in adult patients and is still a matter of debate. Treatment of CHB is not well established and needs to be assessed carefully. The risks and benefits of prenatal fluorinated steroids are discussed.

Adult↗
PubMed1978

Foamy myocardial transformation of infancy: 'lipid' or 'histiocytoid' myocardiopathy.

Two cases of a distinctive infantile myocardiopathy were studied by light and electron microscopy. They represent the 15th and 16th reported examples of this condition, and include the first associated with morphologically classical endocardial fibroelastosis. The condition is characterized by the presence of clusters of foamy or granular cells scattered throughout the myocardium. Ultrastructural studies indicate (1) that lipid storage is not a feature of the condition, and (2) that the abnormal fibers are characterized by a reduced number of fibrils and an increased number of mitochondria. It is proposed that the lesion should be called "foamy myocardial transformation of infancy," and it is speculated that the condition is an acquired metabolic disorder, perhaps occurring in patients abnormally predisposed to such an acquisition.

Cardiomyopathies↗
PubMed2000

Late-diastolic forward flow in the aorta induced by left atrial contraction.

Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography.

Aorta↗
PubMed1985

omega-123I-hexadecanoic acid metabolic probe of cardiomyopathy.

The utility of omega-123I-hexadecanoic acid myocardial scintigraphy as a metabolic probe of cardiomyopathies was investigated. Sixteen patients with a variety of cardiomyopathies and myopathies that involve cardiac muscle and ten volunteers were imaged in the postabsorptive state in a 40 degrees LAO projection after a standard dose of omega-123I-hexadecanoic acid. An elimination T1/2 was calculated from the left ventricular myocardial time-activity curve. An uptake index, corrected for chest wall attenuation, was also computed in 7 of 10 volunteers and 8 of 16 patients. Of the 16 patients, only 2 had distinctly abnormal omega-123I-hexadecanoic acid myocardial tracer kinetics. The first patient had a metabolic disorder of which carnitine deficiency was one component. The second patient had endocardial fibroelastosis, a process which has been linked to disorders which deprive the myocardium of oxygen and energy. Therefore, the cardiomyopathy may have been caused by some abnormality of cardiac metabolism other than carnitine deficiency. Although of limited utility in the overall cardiomyopathic population, omega-123I-hexadecanoic acid myocardial scintigraphy should be further investigated as a screening test for carnitine deficiency and related metabolic abnormalities in patients at risk.

Adolescent↗
PubMedDate not supplied

[Heart transplantation in infants--a new option].

Nowadays orthotopic heart transplantation is world wide accepted as a therapeutic concept in endstage congestive heart failure. Concerning infants, there are still major objections against this kind of therapy and especially in our country exists only very limited experience. Since march 1988 we have performed an orthotopic heart transplantation in 10 pediatric patients: 5 infants (2-16 years of age) with congestive cardiomyopathy, 1 newborn with endocardial fibroelastosis, 3 newborns with hypoplastic left heart syndrome, and finally 1 infant at the age of 2 years with AV-canal and hypoplastic left ventricle. Until now 6 infants survived and are all at home and in good clinical condition. There have been no signs for chronic rejection or graft atherosclerosis up till now. As demonstrated in the example of the newborn, in whom we performed the first successfully orthotopic heart transplantation in Germany, the clinical course and quality of life are superior to those after other palliative procedures in complex cardiac malformations.

Adolescent↗
PubMed1987

Cardiac transplantation in children and adolescents.

Cardiac transplantation represents an expanding therapeutic modality for end-stage heart disease in children and adolescents. During the past 5 years, 27 patients (15 boys; 12 girls) between the ages of 2 and 18 have undergone cardiac transplantation. The preoperative diagnosis was cardiomyopathy in 24 (six familial), congenital heart disease in two, and endocardial fibroelastosis in one. Immunosuppression included cyclosporine, azathioprine, and prednisone. There were 22 survivors, with four hospital deaths (three infection, one pulmonary hypertension), and one death at 4.5 years from graft atherosclerosis. The actuarial survival at 4 years was 83 +/- 7.4% and that at 5 years was 69 +/- 14.2%. Renal function was stable at 4 years, with an average creatinine clearance of 69.75 +/- 27.0 ml/min/m2. Hypertension was present in 21 of 22 patients, who require multiple drug therapy. Rehabilitation is 100% among discharged patients, with 14 in school, six employed, and two toddlers.

Adolescent↗
PubMed1987

[Endomyocardial biopsy in childhood--experiences in 60 pediatric patients].

In 60 pediatric patients, aged from 1 month to 22 years (median 3.54) and a bodyweight of 3 to 67 kg (median 12.6 kg) transvascular endomyocardial biopsy was performed from the right (35 patients) or left ventricle (30 patients). The specimens were investigated by light and electron microscopy. Immune serological investigations were performed in 22 patients, immune histological examinations in eight. There were three indications for biopsy: a. 29 children had a poorly functioning, dilated left ventricle. Of these, seven suffered from endocardial fibroelastosis, 16 from dilated cardiomyopathy, six (plus one control-biopsy) from healing/healed or chronic myocarditis. b. 17 children showed inadequate left ventricular hypertrophy. Of these, ten suffered from HCM, four from secondary hypertrophy, three from storage diseases. c. Various questions were answered in eight children - four with hypoxic, two with cytotoxic myocardial damage. There were five misindications, retrospectively. We observed no serious complications. Evaluation of biopsy revealed diagnostic findings in 11.7%, was helpful in 71.7% and of no help in 16.6%. Hence, even in childhood, endomyocardial biopsy is a diagnostic tool which can contribute useful information on the etiology or pathogenesis of the underlying myocardial disease.

Adolescent↗
PubMed1968

Neonatal cardiac distress. A practical approach to recognition, diagnosis, and management.

The death rate among neonates with cardiovascular disease is 50 percent during the first six months, with the majority dying during the first month. With early diagnosis most of these babies could be saved. In approaching the diagnosis of cardiac distress in the newborn, it is important to remember that the types of cardiovascular disease which cause symptoms and death early in life are quite different from those in older children. Lesions such as hypoplasia of the left heart, transposition of the great arteries, endocardial fibroelastosis, pulmonary atresia, mitral atresia, tricuspid atresia and truncus arteriosus are common, not rare, causes of cardiac distress in the newborn.A classification of neonatal cardiovascular diseases into seven pathophysiological groups is presented as a basis for an effective, practical approach to the differential diagnosis of the potentially lethal lesions. This approach is simplified further since over 90 percent of babies with cardiac distress have one of three lesions: (1) Large left-to-right shunt (characterized by the presence of massive plethora on the chest roentgenogram), (2) Large right-to-left shunt (association with intense cyanosis) or (3) Severe obstruction (including hypoplasia of the left heart, which is the most common cause of death due to cardiac distress during the first week of life).

Aortic Valve Insufficiency↗
PubMed1995

Heart transplantation in an 8-month-old girl. 10th anniversary report.

In 1984, Dr. Denton A. Cooley led a surgical team that implanted a cardiac allograft in an 8-month-old girl who had end-stage cardiac disease secondary to endocardial fibroelastosis. At that time, experience with cardiac transplantation in infants was limited, and the long-term effects of the procedure were cause for concern. Ten years later, our patient is a healthy 4th-grade student who enjoys a remarkably normal life. She has grown and developed quite satisfactorily, and her heart has enlarged in proportion to her overall somatic growth. Long-term immunosuppression has produced no adverse effects, and the child's medical problems have differed little from those of her peers. This landmark case has yielded preliminary answers to a number of important questions about cardiac transplantation in infants and has confirmed our original opinion that the procedure is well warranted in selected patients.

Child↗
PubMed1986

Infarct expansion: pathologic analysis of 204 patients with a single myocardial infarct.

The reasons for the marked variability in expansion of myocardial infarcts are unknown. To examine this question, the hearts in 204 patients with a single myocardial infarct, autopsied at The Johns Hopkins Hospital and studied after coronary arteriography and fixation in distension, were reviewed. There were 58 (28%) hearts with marked infarct expansion, 34 (17%) with moderate expansion and 112 (55%) with no or minimal expansion. The degree of expansion was greater in larger, more transmural infarcts (p less than 0.001). Infarcts with greater expansion had significantly more endocardial thrombus (p less than 0.001) and endocardial fibroelastosis (p less than 0.01). Larger heart weight and degree of left ventricular hypertrophy had a significant negative correlation with infarct expansion (p less than 0.05). A markedly greater degree of expansion was noted in the 101 infarcts (50%) caused by lesions in the distribution of the left anterior descending coronary artery as compared with the 57 infarcts (28%) secondary to right coronary lesions and the 46 infarcts (23%) in the distribution of the left circumflex coronary artery (p less than 0.001). The results show that expansion is associated with large infarcts but is less marked in hearts with ventricular hypertrophy. Expansion occurs predominantly in infarcts in the left anterior descending coronary artery distribution, that is, regions of the left ventricular myocardium with the greatest curvature. These results suggest that the degree to which an infarct expands may be influenced by the preinfarction thickness of the ventricular wall.

Adult↗
PubMed1986

Prognostic factors in valvotomy for critical aortic stenosis in infancy.

Aortic valvotomy for critical aortic stenosis in infancy has had a high mortality. To determine the factors that influence survival, we reviewed the cases of 24 infants who underwent aortic valvotomy in the first 6 months of life (mean 4 1/2 weeks) for aortic stenosis from 1978 to 1984. Cardiopulmonary bypass was used in all patients. Operative mortality was 21% (5/24), four of the five deaths occurring from low cardiac output. Analysis of preoperative factors affecting survival versus nonsurvival revealed that low ejection fraction (60% +/- 17% in survivors versus 36% +/- 2% in nonsurvivors), high left ventricular end-diastolic pressure (16 +/- 7 mm Hg in survivors versus 30 +/- 14 mm Hg in nonsurvivors), and presence of endocardial fibroelastosis (25% in survivors versus 100% in nonsurvivors) all were predictive of a poor outcome, although the small sample size indicated caution in interpreting results. Factors that did not appear to influence survival included peak systolic gradient (79 +/- 30 mm Hg in survivors versus 60 +/- 15 mm Hg in nonsurvivors) and left ventricular end-diastolic volume (37 +/- 17 cm3/m2 in survivors versus 36 +/- 7 cm3/m2 in nonsurvivors). Four patients with a left ventricular end-diastolic volume below 26 cm3/m2 survived. Postoperative gradients averaged 25 +/- 21 mm Hg at 3.4 +/- 2 years' follow-up in nine recatheterized patients. Ejection fraction of these patients increased from 45% +/- 10% to 70% +/- 11% and left ventricular end-diastolic volume increased from 37 +/- 17 to 58 +/- 5 cm3/m2. Two of 17 patients have required apical-aortic conduits; all other patients are asymptomatic. We conclude that infants with critical aortic stenosis benefit from valvotomy even with impaired left ventricular function and severely reduced left ventricular dimensions and many have nearly normal hemodynamics on late follow-up.

Age Factors↗
PubMed1991

[Clinical aspects and development of dilated cardiomyopathy in infants and children].

PURPOSE: The evaluation of the clinical aspects of the dilated cardiomyopathy (DCM) in infants and children regarding, mainly, to the evolution and prognostic of this disease. PATIENTS AND METHODS: 38 patients divided in two groups: A) 22 infants till 22 (11.60 +/- 6.50) months of age, 15 female, and B) 15 children of 2 to 12 years of age (5.23 +/- 3.13) the majority males (10). A retrospective study was carried out based on the data from the patients's records. It was performed, in all the cases, a clinical, electrocardiographic, echocardiographic (M module and two dimensional echocardiography) and radiologic evaluation. RESULTS: The dyspnea on exercise (included sucking) was the predominant symptom in 15 (65.22%) patients of the A group and 10 (66.67%) of the B group followed by perilabial cyanosis in 7 (30.43%) and 6 (40%) patients, respectively. In the A group the clinical diagnostic hypothesis was inspecific myocarditis (IM) in 12 (52.17%), endocardial fibroelastosis (EFE), in 8 (34.79%), and "idiopathic" dilated cardiomyopathy (IDCM) in 3 (13.04%). In the B group to the diagnostic conclusion of myocarditis was made in 10 patients (66.67%)--5 of them IM--EFE in 3 (20%); and IDCM in 2 (13.33%). The average time of evolution was 5.48 months in the A group and 18.56 in the B group. In the A group the evolution was excellent in 3 (3.04%), good in 10 (43.46%), stable in 2 (8.70%) and bad in 1 (4.35%). In the B group, excellent in 8 (53.33%), good in 2 (20%) stable in 1 (6.67%). No bad evolution in this group. There was a decrease in the A group (4.34%); 6 patients in this group (26.09%) and 3 (20%) of the B group interrupt the follow-up. CONCLUSION: 1) The prognosis of infants with DCM including those with the diagnostic hypothesis of EFE seems to be less adverse than it could be supposed to be; 2) the prognostic in children with the diagnosis of DCM established above 2 years of age seems to be good; 3) the differential clinical diagnosis between EFE and IM is difficult and with no accuracy; 4) it is possible that the IM could be more prevalent in infants till 6 months of age than we suppose it was.

Cardiomyopathy, Dilated↗
PubMed1989

Coronary arteries in the hypoplastic left heart syndrome. Histopathologic and histometrical studies and implications for surgery.

The subepicardial coronary arteries (CAs) in the hypoplastic left heart syndrome (HLHS) were studied for pathology dependent on the patency of the mitral valve and the presence of endocardial fibroelastosis (EFE) of the left ventricle (LV). Nine heart specimens with aortic and mitral atresia (group 1) were studied and compared with 19 hearts with aortic atresia and mitral stenosis (group 2) (EFE of the LV was present in all cases) and 10 normal hearts. The median age at death was 3 days. For the histological and histometrical studies, 5-mm blocks from six comparable sites of the left (L) and right (R) CAs were serially sectioned and routinely processed. The arterial size and wall thickness were measured. Macroscopically, the CAs in group 1 were normal except for the prevalence of left dominance (56%). In contrast in group 2, the CAs were thicker and tortuous. Histopathology was restricted almost exclusively to group 2 specimens (11 of 19), with the proximal LCAs being the most affected. However, eight hearts with EFE in group 2 had no structural wall changes of the CAs. Histometrically, the sizes (external diameters) of the LCAs and RCAs were similar (0.5-1.0 mm) in groups 1 and 2 and in the normal group; however, we noted an increase in percent medial thickness in group 2 specimens, especially of the LCAs. Furthermore, in some group 2 hearts, the wall thickness (intima and media) relative to the luminal size (internal radius) of proximal and middle LCAs was significantly increased, suggesting the presence of ventricle-CA communications.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve↗
PubMed1998

Clinical experience with heart transplantation in infants.

OBJECTIVE: Orthotopic heart transplantation has become an accepted therapeutic concept for adult patients with endstage heart disease. In newborns and infants this procedure is still a matter of discussion because of unknown long-term results and the lack of donor organs. METHODS: Since March 1988 we have performed 40 orthotopic heart transplantation in 39 infants who were from 1 to 280 days of age. Indications for transplantation included hypoplastic left-heart syndrome (n = 28), dilative cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and other complex structural anomalies (n = 3). The mean waiting period for transplantation was 53 days. A donor-recipient weight ratio up to 4.0 was accepted. Profound hypothermic circulatory arrest was used for graft implantation in all those patients who required extensive aortic arch reconstruction (71%). The initial immunomodulation was based on Cyclosporine, Azathioprine and Prednisolone. Patients who underwent transplantation during the first 6 weeks of life received a chronic single-drug therapy with Cyclosporine after 1 year. RESULTS: There were six peri-operative deaths caused by drug-resistant right-heart failure in three cases, humoral rejection (n = 1), CMV infection (n = 1) and multi organ failure (n = 1). One infant died late, due to rejection. The actuarial survival rate for the entire group is now 82%. There is a remarkable influence of increasing experience. Whereas six of 15 infants who had heart transplantation between 1988 and 1993 died early post-operatively (survival rate: 60%), only one late death occurred among 24 recipients in the period from 1994 to April 1997 (survival rate: 96%). Episodes of rejection occurred once or several times in about half of the patients in this series (48%). All surviving children are living at home in excellent condition. CONCLUSIONS: Heart transplantation during early infancy is a rational and durable therapy for heart diseases with irreversible myocardial failure or severe structural anomalies. The intermediate-term results have been encouraging in many centers, but more data must be accumulated to determine the sequelae of chronic immunosuppression. The lack of donor organs remains one of the major problems in pediatric heart transplantation.

Body Height↗
PubMed1976

[Postoperative endocardial fibrosis following mitral valve prosthesis].

Uncommon observation of endocardial fibroelastosis of the left auricle and ventricle found at autopsy. The 46-year-old female patient had her mitral valve replaced 15 months ago because of mitral stenosis (and slight incompetence). The left auricle with certainty did not show endocardial fibrosis at the time of operation. The therefore acquired postoperative fibroelastosis of the left auricle is probably caused mechanically resp. by hypoxia since postoperatively valvular incompetence and restenosis with severe thrombosis of the artificial valve developed. There are no reasons to assume an inflammatory etiology. It must remain open if the fibroelastosis of the left ventricle was congenital. Its possible etiology - if it was also acquired - is discussed.

Autopsy↗
PubMed2005

Left ventricular noncompaction: a pathological study of 14 cases.

Left ventricular noncompaction (LVNC) has been recently proposed as a specific form of cardiomyopathy. There have been few pathological series describing gross and microscopic findings of this entity, especially in children. We present findings of 14 hearts (13 autopsy and 1 explant) with LVNC (isolated and associated with congenital heart disease), defined by poorly developed left ventricular (LV) papillary muscles and a noncompact inner LV myocardial layer comprising more than 50% of the LV thickness. The mean age at death/explant was 3.6 years (median, 2.5 months); there were 6 boys and 8 girls. The symptoms were sudden unexpected death (10) and heart failure (4). The diagnosis was suspected before death in only 1 of 13 autopsy cases. Right ventricular involvement (> 75% ventricular thickness comprised of noncompacted area with recess adjacent to tricuspid valve) was seen in 6 of 14 hearts. One patient had a sibling with pulmonary stenosis, but there was no other known familial cardiomyopathy. Endocardial fibroelastosis was a characteristic histological feature, as well as anastomosing or polypoid endocardial trabeculations, which resulted in staghorn-shaped, endocardial-lined recesses. There was a high rate of other cardiac anomalies, which often coexisted and were not clearly related to the LVNC, present in 8 cases (nonisolated LVNC): ventricular septal defect (4/14), anomalous venous pulmonary veins (1/14), coronary ostial stenosis (1/14), histiocytoid cardiomyopathy (1/14), polyvalvar dysplasia (2/14), and pulmonary stenosis (2/14). In the 6 isolated LVNC, there were 2 malformed atrioventricular valves (1 mitral and 1 tricuspid), which appeared part of the ventricular maldevelopment. There were no differences in histological or gross patterns of the noncompacted regions between the isolated and nonisolated LVNC. LVNC is frequently associated with other cardiac defects, especially when causing sudden death in infants and children. A clear-cut morphological distinction between "isolated" and "secondary" LVNC was not apparent. The pathologist should be aware of the entity because the diagnosis is often first established at autopsy.

Autopsy↗