Endocardial Fibroelastosis Resection: When it Works and When it Does Not.

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.

Ethical considerations of transparency, informed consent, and nudging in a patient with paediatric aortic stenosis and symptomatic left ventricular endocardial fibroelastosis.

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.

[Prenatal diagnosis of fetal aortic stenosis with mitral insufficiency. Review of the ultrasound diagnosis and perinatal prognosis: a case report]. / Diagnóstico prenatal de la estenosis aórtica crítica con insuficiencia mitral. Revisión del diagnóstico ecográfico y pronóstico perinatal. A propósito de un caso.

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.

Infant Ross-Konno, Endocardial Fibroelastosis Resection and Mitral Valve Repair.

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.

Combined Aortic and Mitral Annular Enlargement With Fibrous Skeleton Reconstruction.

In a pediatric population, congenital or acquired disease of the aortic and mitral valves may coexist and sometimes require replacement of both valves. Enlargement of aortic and mitral annuli may also be required. We demonstrate a challenging case that required upsizing of both prosthetic valves by redo anterior aortoventriculoplasty and patch enlargement of the aortic-mitral fibrous body. This case highlights the complexity and feasibility of enlarging both annuli in a reoperative setting, to implant larger prostheses.

Electrical dyssynchrony and endocardial fibroelastosis resection in the rehabilitation of hypoplastic left cardiac syndrome.

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.

Prognostic Value of Global Longitudinal Strain and Etiology After Surgery for Primary Mitral Regurgitation.

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.

Flow disturbances and progression of endocardial fibroelastosis - a case report.

Endocardial fibroelastosis (EFE) is described as thickening of the endocardium and is associated with hypoplastic left heart syndrome (HLHS). The stimulus for EFE and the mechanism for recurrence and/or progression need to be investigated. In this report, we describe the case of a 4-year-old HLHS patient who underwent several surgeries with EFE resections due to recurrence of EFE. EFE recurrence was associated with flow disturbances due to valvar defects. At her latest follow-up 7 months after the last surgery, competent valves and no EFE were identified on all imaging study.

Outcomes of mitral valve repair for mitral regurgitation due to degenerative disease.

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.

Surgical approach to cardiac papillary fibroelastomas.

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 which occurred from the posterior leaflet of the mitral valve; report of a case].

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.

A case of cardioembolic stroke due to intracardiac papillary fibroelastoma evaluated by using transesophageal echocardiography.

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.

Unusual case of an 18-year-old heart transplant recipient with endocardial fibroelastosis.

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.

Papillary fibroelastoma of the ascending aorta presenting with cardiogenic shock.

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.

Successful Biopsy and Removal of a Tricuspid Valve Papillary Fibroelastoma in Cardiac Catheterization Laboratory: A Case Report.

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.

Usefulness of magnetic resonance imaging of left ventricular endocardial fibroelastosis in infants after fetal intervention for aortic valve stenosis.

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.