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PubMed2006

Twenty years experience of surgical aortic valvotomy for critical aortic stenosis in early infancy.

OBJECTIVE: To examine early and long-term results of surgical aortic valvotomy in neonates and infants aged less than 3 months. METHODS: A review of all 34 neonates (n=26) and young infants (n=8) aged 1-62 days undergoing primary open valvotomy for aortic valve stenosis between 1983 and 2003 was carried out. Associated major cardiac anomalies were endocardial fibroelastasis (n=8), aortic coarctation (n=3), subvalvular aortic stenosis (n=2), and ventricular septal defect (n=1). Risk factors for early mortality were estimated. Current information was available for 31 patients for a follow-up of 115+/-67 months. Kaplan-Meier method was used to estimate freedom from reintervention. RESULTS: Two neonates died early: operative mortality of 6% (2/34). Risk factors for early mortality were associated endocardial fibroelastosis, monocuspid aortic valve and impaired left ventricular function. No patient died late. Seven patients needed reintervention for re-aortic stenosis (n=5) or aortic insufficiency (n=2), i.e., re-valvotomy (n=3), valve replacement (n=2), Ross procedure (n=1), and balloon valvuloplasty (n=1). Freedom from reintervention was 85.1+/-6.9%, 78.0+/-9.35%, and 53.5+/-15.9% at 5, 10, and 15 years, respectively. CONCLUSIONS: Primary surgical aortic valvotomy in early infancy carries a low early and late mortality, a low occurrence of significant aortic regurgitation and a low early recurrence of aortic stenosis. In great majority of cases, reintervention can be delayed to allow implantation of an adult-sized prosthesis, when required.

Aortic Valve↗
PubMed2005

Neonatal isolated critical aortic valve stenosis: balloon valvuloplasty or surgical valvotomy.

BACKGROUND: Open surgical valvotomy and transcatheter balloon valvuloplasty are recognised treatments for neonatal critical aortic stenosis. METHODS: A retrospective analysis was undertaken of all newborns with critical aortic valve stenosis between 1990 and 2000 presenting to a tertiary centre and who required intervention. The initial catheter and surgical intervention was generally based on the preference of the attending cardiologist and the anatomy of the aortic valve and in consultation with the cardiothoracic surgeon. The two groups were therefore not strictly comparable. Twelve were subjected to balloon valvuloplasty and thirteen to surgical valvotomy at a median age of 11 days (2-42 days) and 3.5 days (1-19 days) respectively. There was no significant difference in the timing of the procedure, weight of the infant, aortic annulus or left ventricular dimensions in either group. RESULTS: There was one unrelated hospital death in the balloon group compared to two in the surgical group both of whom had endocardial fibroelastosis. Mild to moderate aortic regurgitation was seen after both procedures. Four patients in the balloon valvuloplasty group, developed femoral artery thrombosis and two had cardiac perforation that resolved with non operative management. The mean Doppler gradient was reduced from 44+/-14 mmHg to 13.4+/-5 mmHg (p<0.01) in the valvuloplasty group compared to a reduction from 42+/-15 mmHg to 27+/-8 mmHg (p<0.05) in the surgical group. Five patients in the balloon group required re-intervention within 3 weeks to 21 months after the initial procedure. Two patients in the surgical group required a pulmonary autograft and Konno Procedure 3 and 5 years following surgical valvotomy. CONCLUSION: Both aortic valvuloplasty and valvotomy offered effective short and medium term palliation. Balloon valvuloplasty patients had a higher re-intervention rate but shorter hospital and intensive care stay, reduced immediate morbidity and were associated with less severe aortic regurgitation.

Aortic Valve↗
PubMed2006

Congenital complete heart block and maternal connective tissue disease.

Congenital complete heart block can be isolated or can occur in association with other structural heart diseases. Isolated congenital complete heart block (CCHB) is due to transplacental transfer of autoantibodies from mothers with connective tissue disease. Congenital heart block is usually complete, but incomplete blocks, sinus bradycardia and QTc prolongation are also reported. Anti SS A and Anti SS B antibodies transferred from mothers have inflammatory and arrhythmogenic effects in the fetal conduction system. Cardiac manifestations reported include dilated cardiomyopathy, endocardial fibroelastosis and mitral insufficiency. Low ventricular rate, QT prolongation and arrhythmias on monitoring are high risk features. CCHB has a mortality of 30%, and 60% of infants require pacemaker therapy. Fetal echocardiography is useful in early diagnosis. Prophylactic steroid therapy is controversial. Beta adrenergic agonists were tried in mothers with fetuses having congenital heart block to increase fetal heart rate. Early pacemaker therapy is indicated in patients with symptomatic bradycardia and ventricular dysfunction. The indications for pacing in congenital heart block continue to evolve with advances in techniques and most of these children will ultimately require permanent pacemakers by adulthood.

Autoantibodies↗
PubMed2006

Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis.

OBJECTIVES: The purpose of this study was to validate and re-evaluate our previously reported scoring systems for predicting optimal management in neonates with aortic stenosis (AS). BACKGROUND: In 1991, we reported a multivariate discriminant equation and an ordinal scoring system for predicting which neonates with AS are suitable for biventricular repair and which are better served by single ventricle management. METHODS: Retrospective analysis was performed to: 1) validate our scoring systems in 89 additional neonates with AS and normal mitral valve area, 2) assess the effects of 5% measurement variation on predictive scores, 3) evaluate our cohort with the Congenital Heart Surgeons' Society scoring system, and 4) repeat the discriminant analysis on the basis of all 126 patients. RESULTS: The original scores each predicted outcome accurately in 68 patients (77%). Minor (5%) measurement variation changed the outcome predicted by the discriminant equation in 8 patients (9%) and by the threshold system in 13 patients (15%). The most accurate model for predicting survival with a biventricular circulation among the full cohort is: 10.98 (body surface area) + 0.56 (aortic annulus z-score) + 5.89 (left ventricular to heart long-axis ratio) - 0.79 (grade 2 or 3 endocardial fibroelastosis) - 6.78. With a cutoff of -0.65, outcome was predicted accurately in 90% of patients. CONCLUSIONS: Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.

Aortic Valve Stenosis↗
PubMed2004

The Contegra conduit in the right ventricular outflow tract induces supravalvular stenosis.

OBJECTIVE: We sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract. METHODS: Between May 2000 and September 2002, 58 Contegra conduits (8-22 mm) were implanted during primary (n = 27) or redo operations (n = 31) in 57 patients, with ages ranging from 2 days to 48 years (mean, 9 years). Indications were truncus arteriosus (n = 16), tetralogy of Fallot (n = 28), pulmonary replacement in the Ross operation (n = 10), and Rastelli-type repair for double-outlet right ventricle (n = 4). Echocardiography was prospectively performed by a fixed team of investigators during follow-up (mean, 22.7 +/- 10 months). A peak gradient of greater than 50 mm Hg was considered severe stenosis. RESULTS: Two patients died from Staphylococcus aureus -induced septicemia and enterococcal endocarditis after 12 days and 12 weeks, respectively. One patient died of heart failure caused by endocardial fibroelastosis after 1 year. Freedom from severe stenosis at the distal anastomosis was 91% +/- 3% at 3 months, 68% +/- 6% at 12 months, and 49% +/- 8% at 24 months. The risk of development of stenosis does not change over time. Younger age and its derivatives (graft size and indication) are significantly related to the occurrence of severe stenosis ( P < .0001). Seventeen (29%) conduits required an endovascular intervention (balloon dilatation or stent). Seven (12%) conduits were explanted (endocarditis, 2; stenosis, 5). Histologic analysis of the explanted conduits showed excessive proliferation of neointima at the level of the distal anastomosis. Valve regurgitation was observed in 9 (16%) conduits and was always secondary to dilatation in the presence of severe distal stenosis. CONCLUSION: The Contegra conduit induces a neointimal proliferation at the level of the pulmonary anastomosis. This leads to a high incidence of severe stenosis at intermediate-term follow-up.

Adolescent↗
PubMed1976

The clinical course of primary myocardial disease in infants and children.

A total of 161 infants and children, ranging in age from 1 day to 17 years at initial encounter (mean, 3.7 years), was seen over a 30 year period with primary myocardial disease (idiopathic myocarditis, nonobstructive cardiomyopathy, endocardial fibroelastosis, and an anatomically unknown category). These patients were observed from 1 hour to 23 years after initial encounter and cardiac disease has resolved in 27 per cent, resulted in death in 35 per cent, and continues in 38 per cent. The majority were first referred to us with congestive heart failure; all exhibited ST-T changes and cardiomegaly, 67 of 150 had left ventricular hypertrophy, 23 of 151 arrhythmias, and 55 of 153 pulmonary vascular congestion. Initial ventricular depolarization abnormalities were very frequent. Significant clinical predictors of fatal outcome included pulmonary vascular congestion, "northwest" axis deviation, and a cardiac index less than three L./min./M.2. Death occurred during the first year after initial encounter in 44 of 57 who died, and in all 13 with proved myocarditis. Primary myocardial disease is a serious disease of infancy and childhood, resulting in death or residual cardiac disease in three fourths of those affected.

Adolescent↗
PubMed1998

Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants.

BACKGROUND: Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era. METHODS: From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques. RESULTS: Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 +/- 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% +/- 6% at 1 month, 78% +/- 9% at 1 year, and 73.4% +/- 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% +/- 3% at 1 month, 73% +/- 9% at 1 year, and 55% +/- 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 +/- 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 +/- 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients. CONCLUSIONS: Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis.

Actuarial Analysis↗
PubMed1984

Generalized arterial calcification of infancy: three case reports, including spontaneous regression with long-term survival.

Generalized arterial calcification in infancy is a rare disorder in which death usually occurs in infancy, the diagnosis generally being made at autopsy. Three patients are reported. The diagnosis was made during life in two, enabling new information to be collected. Cardiac catheterization in one provided evidence suggesting stiffness of pulmonary and systemic arterial walls. Another is a long-term survivor with spontaneous regression of calcification. The third case, diagnosed at necropsy, was associated with endocardial fibroelastosis.

Aortic Diseases↗
PubMed1995

The Norwood operation and subsequent Fontan operation in infants with complex congenital heart disease.

From April 1987 to September 1993, 60 infants underwent a Norwood operation for complex congenital heart disease including hypoplastic left heart syndrome (n = 41), ventricular septal defect and subaortic stenosis with aortic arch interruption/severe coarctation (n = 7), complex single right ventricle with subaortic stenosis (n = 8), critical aortic stenosis with endocardial fibroelastosis (n = 2), and malaligned primum atrial septal defect with coarctation (n = 2). Age at operation ranged from 1 day to 3.9 months (mean 9 days, median 3.5 days). The operative mortality (< 30 days) was 33% (20 patients). Late mortality was 17% (10 patients). Nine of the 20 (45%) operative deaths occurred during the first 2 days after the operation as a result of sudden hemodynamic instability. All four infants with premature closure of the foramen ovale had pulmonary lymphangiectasia and died of pulmonary failure. Seven operative deaths have occurred in 36 patients since 1990 (19%); in the past 2 years, no operative deaths have occurred in 22 patients. Overall, there are 30 long-term survivors (50%). Twenty-one of these 30 infants have undergone a two-stage repair with a modified Fontan operation at 7.3 to 27.6 months of age (mean 18.1 months) with no mortality. Six patients have entered a three-stage repair strategy by undergoing a hemi-Fontan procedure at 6.8 to 23.0 months (mean 8.8 months) with no mortality, and two of these patients have now had their modified Fontan operation at 23.0 to 46.7 months of age with no mortality (four are still awaiting surgery). Two patients have undergone a two-ventricle repair with a Rastelli procedure, with no mortality at 7.4 and 14.1 months of age. Early in our experience, infants undergoing the Norwood operation had a high early mortality most often related to sudden hemodynamic instability. After we instituted a protocol that adds carbon dioxide to the inspired gas during postoperative mechanical ventilation, the postoperative course became more stable and there have been no operative deaths. In summary, the operative mortality for the Norwood operation continues to improve. A subsequent Fontan operation can be performed with excellent clinical results.

Cardiac Surgical Procedures↗
PubMed1996

Aortic valve replacement with a pulmonary autograft in infants with critical aortic stenosis.

Seven patients with critical aortic stenosis underwent aortic valve replacement with a pulmonary autograft (the Ross operation) between the ages of 5 weeks and 9 months. The operation was considered mandatory for survival because of continued severe heart failure or valve avulsion. Six of the patients had undergone unsuccessful previous palliations, such as commissurotomy, balloon dilation, and transventricular valvotomy, performed singly (n = 1) or in combination (n = 5). The other patient with a severely hypoplastic aortic valve ring underwent the Ross procedure as a primary operation. Two operative deaths occurred. In both cases severe endocardial fibroelastosis was detected at autopsy. One late death 1 year after the operation resulted from progressive hypertrophic cardiomyopathy and pulmonary hypertension. The rest of the patients are doing well, without medications. Apart from trivial regurgitation in two patients, the pulmonary autograft is performing well.

Aortic Valve↗
PubMed1999

Extended aortic root replacement with aortic allografts or pulmonary autografts in children.

OBJECTIVES: To evaluate the early results and effectiveness of left ventricular outflow tract enlargement with aortic allograft or pulmonary autograft in children with complex left ventricular outflow tract obstruction. METHOD: The records of 30 children who underwent aortic root enlargement and replacement with either an aortic allograft (22 patients) or pulmonary autograft (8 patients) between January 1987 and June 1997 were reviewed. The predominant diagnosis was complex left ventricular outflow tract obstruction (n = 19), associated with aortic incompetence in 11 children. Before root enlargement, 27 children underwent surgical valvotomy (14 patients), balloon dilatation (10 patients), or both interventions (3 patients). Mean age at root enlargement was 5.4 +/- 3.5 years (range, 2 days-16 years). Most of the children (27 patients) underwent a Konno aortoventriculoplasty. Concomitant septal myectomy was performed in 4 children, mitral valve procedure in 5 children, and endocardial fibroelastosis resection in 1 child. RESULTS: Five children (17%) died in hospital. Four of these were infants less than 2 months old. All had acute aortic incompetence as the result of recent intervention necessitating urgent operation. The fifth child, aged 10 years, died of myocardial failure 2 weeks after the operation. During the follow-up period (mean length, 4.1 +/- 2.8 years), sudden death occurred in 1 child 3 months after the operation. Follow-up echocardiograms (obtained for 23 of the surviving 24 children within 3 +/- 2.3 years) showed a left ventricular outflow tract gradient reduced from a mean of 65 to 11 mm Hg (P =.001); Z value increased from a mean of -0.5 to 4.1 (P <. 001), and aortic incompetence was trivial or mild except in 2 children. CONCLUSION: Urgent aortic root enlargement in decompensating neonates carries higher mortality rates. In older children, the early results of root enlargement and implantation of allograft or autograft are good.

Adolescent↗
PubMed2003

Congenital atrioventricular block: histological aspects.

It is known that maternal immunological factors such as systemic disease are involved in the genesis of cardiac conduction problems in the fetus but the histologic changes in the conduction system are less documented. We report the case of a 33-year-old woman with no significant medical history. Her first pregnancy was induced by Clomifene. At 17 weeks of gestation, the fetus presented sonographic abnormalities characteristic of a complete atrioventricular block. Biological investigations found anti-SSA and -SSB antibodies. Clinical history search for systemic disease was positive for photosensitivity, lasting 10 years, suggesting the diagnosis of systemic lupus erythematosus. The patient was treated with prednisone 20 mg per day but fetal death occurred at 29 weeks of gestation. Histological examination of the fetal heart showed an altered atrioventricular node and bundle of His with fibrosis, calcifications, endocardial fibroelastosis and mononucleated inflammatory cells. The search for these specific lesions can be determinant in establishing the disease pathogenesis; also, it is important to eliminate this diagnosis in an unexplained fetal death.

Female↗
PubMed1998

Histopathology of the conduction system in sudden infant death.

Because ventricular fibrillation or other arrhythmia have been suggested as a cause of unexplained sudden infant death, many authors focused their studies on the conduction system especially in the 1970s. These studies were not conclusive and many pathologists have abandoned the study of the conduction system. We have reviewed the conduction system of 87 sudden infant death cases between 15 days and 1 year old: 55 were SIDS cases; 17 had mild lesions insufficient to explain death and 15 cases were sudden explained deaths considered as controls. Fetal dispersion of the AV node and/or His bundle and left-sided His bundle, the most frequent findings in the three groups, can be considered normal variations. Nodoventricular tracts were observed in five cases from the three groups whereas fasciculoventricular tracts were present in seven cases of the SIDS group, one case of the second group and in no controls. We consider the possible pathologic significance of this accessory pathway in some SIDS cases. Other infrequent lesions were fibromuscular hyperplasia of AV node artery and His bundle hemorrhage in one SIDS case each; lymphocytic infiltration of the conduction system in one SIDS case and one case with myocarditis and left bundle branch fibrosis in a female infant with endocardial fibroelastosis.

Female↗
PubMed2003

Centronuclear myopathy and cardiomyopathy requiring heart transplant.

Centronuclear myopathy has been extremely rarely associated with cardiomyopathy, which can lead to heart failure and premature death. We report the case of a 3.5-year-old girl with early-onset dilated cardiomyopathy, biventricular hypertrophy and histologic features suggestive of centronuclear myopathy. After unsuccessful medical treatment for heart failure, she underwent cardiac transplantation at the age of 4.5 years. Results of a skeletal muscle biopsy showed increased central nuclei and perinuclear vacuolations with aggregates of mitochondria. Examination of the heart at the time of transplantation confirmed a diagnosis of dilated cardiomyopathy. Histologic results revealed hypertrophic myocardiocytes, focal areas of infarction and endocardial fibroelastosis, most prominently in the left ventricle. Although cardiomyopathy is commonly associated with other childhood myopathies, to our knowledge, this is the youngest patient reported with centronuclear myopathy presenting with heart failure caused by cardiomyopathy, and the first patient to successfully undergo cardiac transplantation. One year after the heart transplant, there were no signs of rejection. We recommend detailed cardiac assessment with regular follow-up for children with histologic features consistent with centronuclear myopathy.

Cardiomyopathies↗
PubMed2002

Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution's experience of 30 years.

OBJECTIVES: We reviewed our institution's experience with isolated (congenital) third-degree atrioventricular block (CAVB) to identify pre- and post-natal predictors of mortality and the requirement for pacemakers in infancy and childhood. BACKGROUND: Because of the relative rarity of the disease, there is a paucity of data concerning the outcome of fetuses and children with isolated CAVB. METHODS: The medical records of all cases of CAVB encountered at our institution from January 1965 to December 1998 were analyzed. RESULTS: Of 102 cases identified, 29 were diagnosed in utero (F) at 26.1 +/- 5.6 weeks gestation, 33 as neonates (N; < or = 28 days), and 40 as children (C) at 5.7 +/- 4.8 years of age. Anti-Ro and/or anti-La were present in 95% of F and 90% of N, but only in 5% of C mothers tested (p < 0.0001). Patients with CAVB having F, N and C diagnosis had a mortality of 43%, 6% and 0%, respectively, in the first two decades of life. Increased mortality risk was associated with a fetal diagnosis of CAVB (13/15 deaths; p < 0.05), fetal hydrops (6/6 cases; p < 0.0001), endocardial fibroelastosis (5/5 cases; p < 0.0001) and delivery at < or = 32 weeks (4/6 cases; p < 0.05). Timing of pacemaker implantation differed significantly among F versus N (p < 0.05) and N versus C (p < 0.001) cases. At 20 years of age only 11% and 12% of CAVB patients with N and C diagnosis, respectively, were not paced. CONCLUSIONS: Pre-natal diagnosis of CAVB is associated with high fetal and neonatal mortality. Among survivors, whether the diagnosis is made before or after birth, most undergo pacemaker implantation by adulthood, with earlier intervention and a significantly greater need for reintervention among those diagnosed in utero.

Age Factors↗
PubMed1985

Idiopathic dilated cardiomyopathy in the young: clinical profile and natural history.

The clinical profile and course of documented cases of idiopathic dilated cardiomyopathy in children have been poorly characterized. Twenty-four patients (median age 2 years, range less than 1 month to 18 years) with idiopathic dilated cardiomyopathy were identified from Mayo Clinic records from 1973 to 1982. The most common presentation was congestive heart failure (92% of patients). Echocardiography (22 patients) generally revealed a dilated left ventricle with reduced fractional shortening (mean 14%) and ejection fraction (mean 26%). Two-dimensional echocardiographic evidence of left ventricular thrombus was present in 3 (23%) of 13 patients. Median cardiac index and left ventricular end-diastolic pressure (19 patients) were 2.5 liters/min per m2 and 22 mm Hg, respectively. Myocardial biopsy in eight patients showed nonspecific findings without active inflammation or evidence of endocardial fibroelastosis. On follow-up (mean duration 33 months, range 0 to 149), systemic arterial embolism had occurred in 2 (8%) of 24 patients. Fifteen of 24 patients had died (63% survival at 1 year and 34% survival at 5 years of follow-up). The cause of death was congestive heart failure in 11, complications after cardiac transplantation in 3 and sudden cardiac death in 1. Nine patients are alive at a mean follow-up time of 65 months (range 26 to 149); five are asymptomatic. Serial determination of left ventricular systolic function, available in all survivors, showed improvement in six patients and no significant change in three. Severe mitral insufficiency was present only in patients who ultimately died. A recent viral syndrome was noted more frequently in patients who survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2001

Clinical spectrum of restrictive cardiomyopathy in children.

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

Adolescent↗
PubMed2000

Sudden cardiac death in infancy due to histiocytoid cardiomyopathy.

Detailed post-mortem is crucial in infants who die suddenly and without a known cause. We report a rare case of histiocytoid cardiomyopathy with endocardial fibroelastosis, the second case in the world literature. The infant presented with sudden death, but the cardiac histological appearance was initially believed to be caused by Pompes disease.

Autopsy↗