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Endocardial fibroelastosis (EFE) is a thickening of the endocardial layer by accumulation of collagen and elastic fibers. Endothelial to mesenchymal transformation is proposed to be the underlying mechanism of formation. Although EFE can occur in both right and left ventricles, this article will focus on management of left ventricular EFE. Through its fibrous, nonelastic manifestation EFE restricts the myocardium leading to diastolic and systolic ventricular dysfunction and prevents ventricular growth in neonates and infants. The presence of EFE may be a marker for underlying myocardial fibrosis as well. The extent of EFE within the left ventricular cavity can be variable ranging from patchy to confluent distribution. Similarly the depth of penetration and degree of infiltration into myocardium can be variable. The management of EFE is controversial, although resection of EFE has been reported as part of the staged ventricular recruitment therapy. Following resection, EFE recurs and infiltrates the myocardium after primary resection. Herein we review the current experience with EFE resection., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Optimal management of critical aortic stenosis (AS) in infants depends on the left ventricle's (LV's) ability to maintain adequate output. Determining feasibility of biventricular repair may be difficult, particularly in those with mitral disease, endocardial fibroelastosis (EFE), multi-level obstruction, and uncertain physiologic capacity. We report a case of a three-month-old with critical AS, severely reduced LV function, EFE, and moderate mitral regurgitation (MR), who underwent a Ross-Konno procedure with concomitant EFE resection and mitral valve repair. Although the technical sequence is challenging, definitive surgery completely relieved multi-level obstruction and MR with markedly improved LV function.

Objectives: This study sought to investigate whether left ventricular (LV) global longitudinal strain (GLS) is associated with long-term outcome after mitral valve (MV) surgery for primary mitral regurgitation (MR) and assess the differences in outcome according to MR etiology: Barlow's disease (BD), fibroelastic deficiency (FED), and forme fruste (FF)., Background: Appropriate timing of MV surgery for primary MR is still challenging and may differ according to the etiology. In these patients, LV-GLS has been proposed as more sensitive measure to detect subtle LV dysfunction as compared with LV ejection fraction., Methods: Echocardiography was performed in 593 patients (64% men, age 65 ± 12 years) with severe primary MR who underwent MV surgery, including assessment of LV-GLS. The etiology (BD, FED, or FF) was defined based on surgical observation. During follow-up, primary endpoint was all-cause mortality and a secondary endpoint included cardiovascular death, heart failure hospitalizations, and cerebrovascular accidents., Results: During a median follow-up of 6.4 (interquartile range: 3.6 to 10.4) years, 146 patients died (16 within 30 days after surgery), 46 patients were hospitalized for heart failure, and 13 patients had a cerebrovascular accident. Age (hazard ratio [HR]: 1.08; 95% confidence interval [CI]: 1.05 to 1.11; p < 0.001) and LV-GLS (HR: 1.13; 95% CI: 1.06 to 1.21; p < 0.001) were independently associated with all-cause mortality. Patients with LV-GLS >-20.6% (more impaired) showed significant worse survival than did patients with LV-GLS ≤-20.6%; of interest, patients with BD showed similar prognosis compared with FED and FF. In addition, previous atrial fibrillation (HR: 1.70; 95% CI: 1.01 to 2.86; p = 0.045) and LV-GLS (HR: 1.01; 95% CI: 1.01 to 1.15; p = 0.019) were independently associated with the secondary endpoint., Conclusions: LV-GLS is independently associated with all-cause mortality and cardiovascular events after MV surgery for primary MR and might be helpful to guide surgical timing. Importantly, patients with BD showed similar prognosis when corrected for age, compared with patients with FED or FF., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

A 62-year-old woman had a prior ischemic stroke in the right temporal lobe with dysarthria and dysesthesia of the left hand. Embolic stroke of undetermined source (ESUS) was diagnosed and warfarin was administered. However, transient ischemic attack recurred upon admission to our hospital. Paroxysmal atrial fibrillation and cerebral arterial stenotic lesions were absent. Transesophageal echocardiography revealed a mobile hyperechoic structure on the aortic valve indicating papillary fibroelastoma. She was diagnosed with a brain embolism due to the intracardiac tumor which was surgically excised and pathologically confirmed as papillary fibroelastoma. This type of tumor is relatively rare but it is important as an embolic source especially in ESUS. Transesophageal echocardiography was indispensable for detecting the embolic source in this patient with ESUS.

A 9-year-old boy who was born with bicuspid aortic stenosis underwent two unsuccessful aortic valvuloplasty interventions, and by 2 years of age he developed restrictive cardiomyopathy caused by left ventricular endocardial fibroelastosis and diastolic dysfunction. The attending cardiologist referred the patient to a high-volume, high-profile congenital cardiac surgical programme 1000 miles away that has a team with considerable experience with left ventricular endocardial fibroelastosis resection and a reputation of achieving good results. Owing to problems with insurance coverage, the parents sought other options for the care of their child in their home state. Dr George Miller is a well-respected local congenital and paediatric cardiac surgeon with considerable experience with the Ross operation as well as with right ventricular endocardial fibroelastosis resection. When talking with Dr Miller, he implied that there is little difference between right ventricular endocardial fibroelastosis and left ventricular endocardial fibroelastosis resection, and stated that he would perform the operation with low mortality based on his overall experience. Dr Miller stated that the local institution could provide an equivalent surgical procedure with comparable outcomes, without the patient and family having to travel out of state. A fundamental dilemma that often arises in clinical surgical practice concerns the conduct of assessing and performing new procedures, especially in rare cases, for which the collective global experience is scant. Although Dr Miller has performed right ventricular endocardial fibroelastosis resection, this procedure differs from left ventricular endocardial fibroelastosis resection, and he cannot be sure that he will indeed be able to perform the procedure better than the high-volume surgeon. This ethical situation is best understood in terms of the principles of respect for patient autonomy, beneficence, non-maleficence, and justice. The tension between the imperatives of beneficence and the obligation to respect the autonomy of the patient by acting only with the patient's best interest in mind is discussed.

Papillary fibroelastomas are rare benign cardiac tumours with a predilection for cardiac valves. Because of the rarity of these tumours, management is individualized, but some recommend surgical removal of all papillary fibroelastomas due to the increased risk of embolization. We report a case of a 71-year-old man who presented with a sessile mass on the tricuspid valve. The mass, a papillary fibroelastoma, was successfully biopsied and removed in the cardiac catheterization laboratory. This report demonstrates a unique minimally invasive way of approaching a cardiac tumour wherein a major surgery was avoided., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

This is a report about a case of prenatal diagnosis of critical fetal aortic stenosis with severe mitral valve insufficiency in a 35+6 weeks fetus. Aortic stenosis represents 3% of congenital heart diseases, but its association with mitral regurgitation is quite unusual. Thanks to the latest advances in fetal ultrasonography we can now achieve a more precise diagnosis and we have been able to improve the understanding of its physiopathology. Based on this case we have reviewed the most recent literature about fetal aortic stenosis and mitral valve insufficiency, with the aim of summarizing its main physiopathological features, highlighting the clues and key points for its intrauterine diagnosis, describing its principal complications and summarizing its current treatment options.

We report a case of a patient who presented with aortic stenosis and a borderline left ventricle during foetal life. A balloon aortic valve valvuloplasty was performed in uterus, and in the postnatal period for relief of the left ventricular outflow tract obstruction followed by a Ross-Konno procedure with fibroelastosis resection. These successful interventions allowed left ventricular growth and the conversion to a biventricular circulation after a single-stage surgery., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Fetal aortic balloon valvuloplasty (FAV) has shown promise in averting progression of midgestation aortic stenosis (AS) to hypoplastic left heart syndrome in a subset of patients. Patients who achieve biventricular circulation after FAV frequently have left ventricular (LV) diastolic dysfunction (DD). This study evaluates DD in fetuses with AS by comparing echocardiographic indices of LV diastolic function in fetuses underwent FAV (n = 20) with controls (n = 40) and evaluates for LV factors associated with DD in patients with FAV. We also compared pre-FAV and post-FAV DD variables (n = 16). Median gestational age (24 weeks, range 18 to 29 weeks) and fetal heart rate were similar between FAV and controls. Compared with controls, patients with FAV had universally abnormal LV diastolic parameters including fused mitral inflow E and A waves (p = 0.008), higher E velocity (p <0.001), shorter mitral inflow time (p = 0.001), lower LV lateral annulus E' (p <0.001), septal E' (p = 0.003), and higher E/E' (p <0.001) than controls. Patients with FAV had abnormal right ventricular mechanics with higher tricuspid inflow E velocity (p <0.001) and shorter tricuspid inflow time (p = 0.03). Worse LV diastolic function (lower LV E') was associated with higher endocardial fibroelastosis grade (r = 0.74, p <0.001), large LV volume (r = 0.55, p = 0.013), and sphericity (r = 0.58, p = 0.009) and with lower LV pressure by mitral regurgitation jet (r = -0.68, p <0.001). Post-FAV, fewer patients had fused mitral inflow E and A than pre-FAV (p = 0.05) and septal E' was higher (=0.04). In conclusion, fetuses with midgestation AS have evidence of marked DD. Worse DD is associated with larger, more spherical LV, with more extensive endocardial fibroelastosis and lower LV pressure., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients., Methods: Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft., Results: Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years., Conclusions: Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The authors report a case of cerebral infarction in a young woman with no obvious risk factors for stroke. The use of effective cardiac imaging enabled a diagnosis of mitral valve papillary fibroelastoma to be made and aided effective surgical management.

Background: Staged left ventricular rehabilitation is a novel surgical approach in patients undergoing single ventricle palliation for borderline hypoplastic left cardiac disease, in an attempt to salvage the left ventricle. The procedure includes resection of endocardial fibroelastosis from the left ventricular free wall and apex. We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics., Methods: This study included 27 patients with borderline hypoplastic left cardiac syndrome who underwent staged left ventricle rehabilitation with endocardial fibroelastosis resection following single ventricle palliation. The effect on electrical synchrony was measured by ventricular depolarisation timing (QRS duration) on electrocardiogram. Patients were evaluated for a change in QRS duration before and after fibroelastosis removal and at most recent follow-up., Results: The QRS change in the immediate period after endocardial fibroelastosis resection ranged from -16 to 36 milliseconds with a median of 0 (p = 0.09). However, long-term conduction delay was common in 44% (12/27) of patients having a QRS duration greater than 98th percentile for the age at the most recent electrocardiogram. Only one patient had QRS duration greater than 98th percentile before any surgical procedure. Two patients developed left bundle branch block and one developed right bundle branch block with left, but anterior-fascicular block. Overall, the QRS duration correlated with left ventricular size (R = 0.54, p = 0.006) at the most recent electrocardiogram., Conclusions: Electrical dyssynchrony is a common finding in patients undergoing staged left ventricular rehabilitation after single ventricle palliation; however, it is not acutely related to surgical endocardial resection. Left ventricular size is correlated with QRS duration. Diligent follow-up is required to evaluate the effects of left ventricular growth and consideration of resynchronisation in this population.

Objective: Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation--endocardial fibroelastosis resection with mitral or aortic valvuloplasty--on left ventricular function and clinical outcomes., Methods: All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test., Results: Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1-38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively., Conclusion: In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.

We describe a case of a young patient admitted for recurrent ischemic stroke caused by a papillary fibroelastoma of the mitral valve and a hyperhomocysteinemia. A papillary fibroelastoma is a benign cardiac tumor which can be associated with serious embolic complications. A moderate plasma level of hyperhomocysteinemia is considered as a risk factor of ischemic stroke. The authors suggest that this association increases the risk of ischemic stroke in their patient. The tumor was surgically removed to avoid new embolic events associated with a vitamin B supplementation. After surgery and acid folic supplementation, no recurrence was observed.

Cardiac papillary fibroelastomas are very rare and benign tumors, usually involving heart valves. They are sometimes asymptomatic. However, they become serious illnesses when associated with clinical events, as systemic embolism. Diagnosis is evoked by echocardiography and confirmed by histopathological examination. The only curative treatment consists of the excision of the tumor under cardiopulmonary bypass. This approach is safe and prevents the risk of embolism recurrence. We report the cases of two patients suffering from acute cerebral embolism. Transthoracic and transoesophageal echocardiography detected tumors of the mitral and aortic valve in the first and second patients, respectively. Preserving the valve integrity, both masses were surgically excised and pathological findings confirmed the diagnosis. As surgical management is curative, we believe that these lesions should be always removed.

Papillary fibroelastoma is a benign primitive cardiac tumour, which can be associated with serious embolic complications. We report on a 37-year-old woman admitted for a cerebrovascular accident. Transthoracic and transesophageal echocardiography revealed a small and pedunculated mass attached to the anterior mitral leaflet. Because of the severity of its symptoms and its high embolic potential, the tumour was surgically excised and histologically diagnosed as a papillary fibroelastoma. The postoperative period was uneventful and the patient was discharged at the third day. Papillary fibroelastoma must be surgically treated because of its high embolic potential and its severe prognosis.

Background: Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. The diagnosis is difficult to establish because clinical symptoms and electrocardiographic findings are nonspecific. Surgical resection of EFE requires the establishment of the diagnosis and delineation of the extent of the fibrotic changes., Objective: To describe the use of MRI in the assessment of EFE in children., Materials and Methods: Three children after surgery for aortic stenosis who were suspected of having EFE were evaluated by echocardiography and MRI. The MR evaluation consisted of black-blood, triple IR, bright-blood, perfusion and myocardial delayed-enhancement sequences. EFE was confirmed at surgery in all patients., Results: Echocardiograms demonstrated vigorous systolic function but substantial diastolic dysfunction of the left ventricle in all. Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. No study was thought to be diagnostic of endocardial fibrosis. On MRI EFE manifested at the endocardial surface as a rim of hypointense signal in the perfusion sequences and as a rim of hyperintense signal in the myocardial delayed-enhancement sequences. The black-blood, triple IR, and bright-blood sequences were not diagnostic., Conclusion: The diagnosis of EFE is difficult to establish by echocardiography. MRI using perfusion and myocardial delayed enhancement can be useful in establishing the diagnosis.

Objective: Durability assessment of mitral valve repair for degenerative valve incompetence is limited to reoperation as a primary indicator and valve-related risk factors for late death as a secondary indicator. We assessed serial echocardiographic follow-up of valve function as an indicator of the durability of mitral valve repair., Methods and Results: In 348 patients having undergone mitral valve repair for degenerative valve incompetence, clinical outcome was excellent: 10 years after repair, survival was 80.1% and freedom from reoperation 94.4%. However, freedom from mitral regurgitation (>2/4), 98.7% at 1 month, decreased to 82.2% at 5 years and 64.9% at 10 years. The linearized recurrence rate of mitral regurgitation (>2/4) was 3.2% per year. Recurrence rate was higher in patients with Barlow disease (6.0%) and lower in those with fibroelastic deficiency (2.6%) (P = .01). Performing chordal shortening, the nonuse of sliding plasty and the nonuse of an annuloplasty ring were determined to be factors predicting recurrence of mitral regurgitation. In reconstructions avoiding these risk factors, recurrence rate decreased to 2.4%. There was no difference between Barlow disease and fibroelastic deficiency: 2.9% versus 2.2% (P > .05). Recurrent regurgitation is characterized by leaflet prolapse, thickening, and calcification., Conclusion: When optimal surgical techniques are used, the residual recurrence rate of mitral valve regurgitation remains between 2% and 3% per year and is related to progressive degeneration of the chordae and the leaflets. Long-term results of mitral valve repair in Barlow disease are essentially the same as in fibroelastic deficiency.

The aim of this study was to review the clinical and echocardiographic outcomes after mitral valve repair for mitral regurgitation due to degenerative disease of the mitral valve. A total of 649 consecutive patients who had isolated mitral valve repair were prospectively followed up for 6.8 +/- 3.1 years. The mean age was 58 +/- 11 years. The operative mortality rate was 0.6%; the late mortality rate was 14.6%; and survival at 15 years was 67 +/- 5%. Age by increments of 5 years, advanced functional class, and impaired left ventricular function were independent predictors of late death. The freedom from reoperation on the mitral valve at 15 years was 92 +/- 3%, and the freedom from late, recurrent, severe mitral regurgitation was 85 +/- 4%. Most patients were in functional classes I or II at the latest follow-up contact. Mitral valve repair is associated with low operative mortality and morbidity, but it does not arrest the degenerative process. This study suggests that rates of reoperation underscore rates of late failure of the mitral valve repair.

The routine use of echocardiography has led to an increase in the diagnosis of cardiac papillary fibroelastomas. From 1990 to 2004, 10 cases of papillary fibroelastoma were observed, nine of which underwent successful surgical excision with valve repair or replacement and without major complications. One patient presented with an asynchronous lesion requiring repeat excision. Surgical excision of papillary fibroelastomas is safe and curative, and carries minimal morbidity. A review of the current literature suggests that symptomatic cardiac papillary fibroelastomas should be surgically removed, whereas asymptomatic lesions that are left-sided, large (larger than 1 cm) or mobile should be considered for surgical excision.

Papillary fibroelastoma is a rare benign tumor arising from the cardiac endothelium. In this report, we describe the surgical treatment for mitral valve papillary fibroelastoma with hypothyroidism. A 69-year-old woman was admitted to our hospital for the treatment of cardiac tamponade. Echocardiography revealed massive pericardial effusion and a small tumor attached to the posterior mitral leaflet. We drainaged the pericardial effusion, and found that the cause of pericardial effusion was hypothyroidism. After controling the thyroid function, open heart surgery was performed. We excised the tumor including a part of the posterior mitral leaflet, and mitral valve plasty was done. Both the surgical and histological findings showed papillary fibroelastoma, and the postoperative course was uneventful. To avoid embolic complications, early surgical intervention is recommended.

We describe an uncommon case of a 58-year-old woman who presented with cardiogenic shock. The echocardiography examination revealed a papillary fibroadenoma located in the ascending aorta which was subsequently surgically treated.

An 18-year-old female Japanese patient who suffered from heart failure and severe pulmonary hypertension was referred to our clinic. The etiology of her cardiomyopathy was unclear. Inhaled prostacyclin therapy resulted in an improvement of pulmonary arterial pressure and allowed us to avoid lung transplantation. Heart transplantation resulted in a complete remission of her respiratory function. Autopsies of the explanted heart revealed massive endomyocardial fibroelastosis. We concluded that endomyocardial fibroelastosis has to be considered a cause of heart failure in young adults with unclear cardiomyopathy.

Primary cardiac tumors are rare. The majority of primary tumors of the heart are benign with papillary fibroelastomas being the third most common after myxomas and lipomas respectively. Papillary fibroelastomas represent the most common valvular tumor of the heart. Although benign, papillary fibroelastomas have the potential to cause turbulent flow, thrombus formation, and severe cerebral or coronary embolic disease. The majority of fibroelastomas have been described on the left side of the heart involving the aortic and mitral valves. We present a rare case of a papillary fibroelastoma occurring on the pulmonic valve.

This report describes the use of myocardial delayed-enhancement magnetic resonance imaging to delineate the extent of endocardial fibroelastosis (EFE) in 4 infants after fetal balloon aortic valvuloplasty. The preoperative mapping of EFE followed by extensive resection led to biventricular physiology in 2 patients.

Cardiac fibroelastomas are rare benign tumours. Initially observed as a chance finding at autopsy or during cardiac surgery, the diagnosis has become more common since the introduction of echocardiography. These tumours are usually asymptomatic. When the left heart valves are involved, embolic complications may be threatening. When the right heart valves are affected they are usually asymptomatic. The authors report the case of a fibroelastoma of the tricuspid valve responsible for several syncopal attacks. The tumour was excised with preservation of the native valve. There has been no recurrence of syncope since surgery. The authors believe this to be the second reported case of fibroelastoma of the tricuspid valve presenting with syncope.

We report the case of a 75-year-old female with atypical chest pain followed by non-Q wave myocardial infarction. At coronary angiography and ventriculography severe left main stenosis and severe mitral regurgitation were evidenced. Transesophageal echocardiography, performed in order to evaluate a possible mitral repair, showed an aortic tumor (11 x 14 mm in diameter) attached to the commissure between the left and non-coronary cusp, without mitral regurgitation. Cardiac magnetic resonance showed a relationship between the tumor and the left coronary ostium which explained a paroxysmal mitral regurgitation. The tumor was surgically removed and the resulting defect in the aortic leaflet was reconstructed with an autologous pericardial patch. The tumor resulted to be a papillary fibroelastoma. The clinical course was uneventful.

Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.

Endocardial fibroelastosis is un uncommon disease and it has a very bad prognosis since fatal evolution is usual before 2 years old. We report the case of a 20 years old woman who is affected with the contracted form of this disease associated with atrial septal defect (ASD) and mitral regurgitation. This disease was discovered by endocardial biopsy when she was 4 years old and underwent surgical resection of endocardial fibrosis, a patch on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy.

Background: Until recently, newborns with medically intractable cardiac failure caused by congenital malformations were mostly doomed to death because of the severity of the disease, which precludes a palliative operation, or because of fatal deterioration before availability of a suitable donor heart., Methods: The recently developed paracorporeal pneumatically driven Medos HIA ventricular assist device offers a therapeutic option for these small infants because it is manufactured in various sizes and is even suitable for cardiac assistance in neonates with a body surface area less than 0.3 m2., Results: We report our initial experience with this device, which we used for univentricular bridging to total orthotopic cardiac transplantation in 3 infants. The device was inserted to support the left ventricle in two instances and to support the right heart in one. Successful bridging to transplantation was achieved in 2 infants for periods of 2 and 7 weeks., Conclusions: Our experience demonstrates the feasibility of univentricular mechanical support followed by successful cardiac transplantation in infants and newborns.

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.