Bidirectional Cavopulmonary Shunt for Right Ventricular Unloading.

BACKGROUND: Right-sided heart failure remains a challenge in the care of congenital heart disease patients, both those with right ventricular dilation and dysfunction and those with right ventricular hypoplasia. Two strategies for treatment are atrial septal fenestration and bidirectional cavopulmonary shunt (BCPS). METHODS: This review details the strategies for right ventricular unloading, with summaries of pertinent data and commentaries on the subject. RESULTS: While atrial septal fenestration provides right ventricular unloading and can be appropriate in cases of moderate right ventricular dysfunction and dilation, this unloading is not as substantial as a BCPS. A BCPS more effectively unloads the right ventricle, provides preload to the left ventricle, and can significantly improve ventricular-ventricular interactions. A BCPS is often appropriate in cases of severe right ventricular dysfunction and dilation, if factors favorable for BCPS circulation are in place. Certain anatomic and physiologic factors assessed both preoperatively and intraoperatively help guide the decision regarding which patient may benefit from right ventricular unloading and which technique is optimal. CONCLUSIONS: When used strategically in select patients, BCPS and atrial-level fenestration are effective in managing right ventricular failure in congenital heart disease patients. Preoperative imaging and intraoperative anatomic and physiologic factors help guide the appropriate management for a given patient.

Cardiac resynchronization therapy improves heart failure in one patient with acromegaly-induced cardiomyopathy: a case report.

BACKGROUND: Congestive heart failure is rarely observed in patients with acromegaly. Excessive growth hormone secretion and elevation of insulin-like growth factor 1 contribute to pathological changes in myocyte growth and structure, cardiac contractility, vascular function, and in later stage may progress to cardiac dysfunction. Early recognition of the condition is paramount, though the insidious progression of the disease commonly results in late diagnosis. Current standard regimens of pharmacological therapy, surgical treatment, radiotherapy are designed to normalize serum levels of both insulin-like growth factor 1 and growth hormone. In patients with late-stage heart failure due to acromegalic cardiomyopathy, cardiac resynchronization therapy might be a desirable treatment to help cardiac synchronization, improve symptoms, and eventually reduce hospital admissions together with mortality rates. CASE PRESENTATION: We describe a case of a 49-year-old man with a history of acromegaly who presented to our hospital with a diagnosis of decompensated systolic heart failure. Serial electrocardiograms showed wide (160-200 ms) QRS duration with left bundle branch block. Echocardiography showed severe left ventricular dysfunction that simultaneously achieved a left ventricular ejection fraction of 16%. Surgical indication was rarely assessed by neurosurgeons. Given that the stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects, cardiac resynchronization therapy was performed. Owing to biventricular synchronization and holding back reverse remodeling, the patient's symptoms were successfully alleviated, and he was discharged from the hospital. CONCLUSIONS: Congestive heart failure is a rare complication in acromegaly-induced cardiomyopathy (occurs in only 3% of patients). Early diagnosis and treatment with curative drugs more than cardiovascular implantable electronic devices might lead to better surgical outcomes in this group of patients.

Improvement of the outcome in patients with infantile dilated cardiomyopathy over three decades - The usefulness of long-term gradually medical supportive care.

BACKGROUND: The treatment of heart failure has changed with the use of angiotensin-converting enzyme inhibitors (ACEIs) and beta-blockers since the middle of the 1990s. However, the outcome in infantile dilated cardiomyopathy (DCM) when treated with them remains poorly understood. METHODS: We reviewed the medical records of infants with DCM within 24 months old in our hospital between 1979 and 2012, and compared the outcome in the later group (1997-2012) with that in the early group (1979-1996). The survival and cardiac event (CE)-free survival rates were calculated by the Kaplan-Meier method. RESULTS: There were 20 patients in the early group and 24 patients in the later group. The median left ventricular fractional shortening at the onset of disease in the early and later groups were 11% (range 4-17%) and 12% (range 4-25%), respectively. In the later group, ACEIs and beta-blockers were administered in 22 and 21 patients, respectively. An usual low-dose induction of carvedilol therapy (0.01-0.02mg/kg/day) sometimes worsened the heart failure in 9 patients (43%) after the successful initial conventional treatment for acute heart failure. Nineteen patients died and 25 survived. The CEs were as follows: heart transplantation 4, mitral valvuloplasty 1, Batista operation with mitral valve replacement 1, and cardiac resynchronization therapy in the late period 1. The 20-year survival rate in the early and later groups were 5% (95% CI 0.7-28) and 100%, respectively (p<0.001). The 2-year CE-free survival rate in the early and later groups were 5% (95% CI 0.7-28) and 83% (95% CI 59-91), respectively (p<0.001). CONCLUSIONS: The outcome in patients with infantile DCM has significantly improved with careful acute and chronic treatments using ACEIs and beta-blockers since the 2000s. Adopting a long-term supportive treatment during a period of low ventricular function and the use of beta-blockers corresponding to each patient's condition were key to survival.

Early severe coronary heart disease and ischemic heart failure in homozygous familial hypercholesterolemia: A case report.

RATIONALE: Familial hypercholesterolemia (FH) is a common inherited cause of coronary heart disease (CHD) and premature death in an early age. Nevertheless, an ischemic heart failure (IHF) associated with FH seems to be rare, and an early diagnosis and therapy could influence the prognosis. PATIENT CONCERNS: In this 13-year-old girl, multiple xanthomas began to develop from the first day of birth. Until June, 2017, she was admitted to our center due to edema, oliguria, and dyspnea during exertion, which was attributed to a recent respiratory infection. DIAGNOSIS: Homozygous FH (HoFH), CHD, and IHF. INTERVENTIONS: The patient has been treated with statin, ezetimibe, aspirin, and traditional heart failure (HF) medications. In addition, the beta-blocker was simultaneously administered. OUTCOMES: Genotypes of this proband indicated homozygous mutations of low-density lipoprotein receptor (LDLR) and some co-segregated mutations, such as von Willebrand factor (VWF) and fibroblast growth factor receptors. At 6-month follow-up, we found a decreased level of plasma lipid profile, in addition to a significant improvement in 6-minute walk distance and functional class. Echocardiography indicated nonsignificant improvements in the structure and function of the heart. LESSONS: This case report indicates that HoFH can lead to dramatically progressive endothelial damages and ventricular remodeling, severe atherosclerosis, even IHF. Genetic outcomes indicate IHF with HoFH could possibly result from LDLR mutations and some co-segregated mutations influencing endothelial function and cardiovascular remodeling. In a short-term follow-up, a combination of statins, ezetimibe, aspirin, and traditional HF agents is safe and effective for IHF with HoFH, and there is a need for further identification of drugs to ameliorate endothelial function and cardiovascular remodeling which may play an important role in long-term treatment.

Dysplastic megalencephaly phenotype presenting with prenatal high-output cardiac failure.

Dysplastic megalencephaly, also known as bilateral hemimegalencephaly, is a rare cerebral malformation characterized by bilateral cerebral hemisphere overgrowth and extensive malformation of cortical development. Affected patients present clinically with intractable seizures, severe neurological impairment and global developmental delay. There is a small body of literature reporting megalencephaly's association with neonatal high-output cardiac failure and a lack of literature describing prenatal findings. We report a case of dysplastic megalencephaly presenting with progressive high-output cardiac failure during fetal life. Prenatal and postnatal imaging findings as well as neonatal course are described. A companion case with similar imaging findings will help illustrate the prenatal imaging characteristics of this association. Knowledge of this potential complication related to dysplastic megalencephaly may help guide parental counseling and obstetric management.

Association Between C1q/TNF-Related Protein-1 Levels in Human Plasma and Epicardial Adipose Tissues and Congestive Heart Failure.

BACKGROUND/AIMS: C1q and tumour necrosis factor-related protein 1 (CTRP1) possesses anti-atherogenic and anti-inflammatory effects. This study investigated whether the CTRP1 levels in the plasma and epicardial adipose tissue (EAT) were associated with congestive heart failure (CHF) and to disclose possible molecular mechanisms. METHODS: Plasma and tissue samples were obtained from subjects with or without CHF. Plasma levels of CTRP1 were measured by ELISA. The mRNA levels of CTRP1 and inflammatory cytokines were detected by RT-PCR. The protein levels of CTRP1, aldosterone synthase (CYP11B2) and mitogen-activated protein kinase were examined by Western blotting. RESULTS: The levels of CTRP1 in the plasma and EAT were higher in the CHF patients than those in the controls. There were no differences in the CTRP1 levels in cardiomyocytes between the CHF group and the non-CHF group. An exploratory survival analysis showed that higher CTRP1 values at admission were associated with a worse prognosis after discharge. CTRP1 increased the IL-6 mRNA level in H295R cells. CTRP1 recruited ERK1/2 and Jak-2 for aldosterone release by modulating the CYP11B2 protein level, and brain natriuretic peptide repressed the CTRP1-induced aldosterone release through the JAK2-STAT3 signalling pathways. CONCLUSION: The CTRP1 levels in the plasma and EAT were increased in the CHF patients. CTRP1 is involved in the pathogenesis of CHF by modulating IL-6 levels and aldosterone release.

Tissue Plasminogen Activator Use in Children: Bleeding Complications and Thrombus Resolution.

OBJECTIVE: To review our institutional experience with tissue plasminogen activator (tPA) to determine outcomes related to bleeding complications and thrombus resolution. STUDY DESIGN: We performed a retrospective review of all patients who received systemic tPA for thrombolysis. Data points included location of thrombus, initial and maximum tPA dose, and duration of tPA. The primary endpoint was bleeding complication. RESULTS: Between 2005 and 2014, 46 patients received systemic tPA for thrombolysis: 17 (37%) were patients with a primary cardiac diagnosis, there were 17 (37%) hematology/oncology patients, and 12 (26%) patients with noncardiac, nonhematology/oncology diagnoses. The indication for tPA was central venous thrombus (n = 23), pulmonary artery thrombus (n = 9), and cardiac or aortic thrombus (n = 14). Bleeding complications occurred in 15 patients (33%). Median initial tPA dose in the bleeding complication group was 0.10 mg/kg/h vs 0.03 mg/kg/h in the group without bleeding complication group (P = .01). Cardiac patients experienced more bleeding complications (P = .01). Multivariate analysis indicated that dose of tPA (P = .01) and diagnostic category (P < .01) were associated with bleeding complication. Complete thrombus resolution occurred in 21 patients, partial in 10 patients, and no resolution in 15 patients. Complete resolution of thrombus was not associated with diagnosis, thrombus location, tPA dose, or duration. CONCLUSIONS: Cardiac patients appear to be at highest risk of bleeding complication; bleeding complications were associated with higher doses of tPA, and cardiac patients were the cohort who received the highest doses of tPA. Higher tPA doses are associated with increased risk of bleeding complication but are not associated with successful thrombus resolution.

Cardiovascular profile score in 44 fetuses with cervicofacial tumors.

BACKGROUND: Cervicofacial tumors are rarely detected by prenatal ultrasound, and prenatal counseling is very difficult as not much information is available about this problem in the literature, other than cases reports. OBJECTIVES: The aim of this study was to know if fetal echocardiography examination is helpful in those cases. MATERIAL AND METHODS: Forty-four fetuses with cervicofacial tumors detected in utero by ultrasonography and referred to our unit were assessed and the cardiovascular profile score (CVPS) based on echocardiography examinations was calculated. The data were analyzed by the standard statistical tests and Pearson's χ2-test (significance level P=0.05). RESULTS: CVPS<8 was diagnosed in 10 fetuses (23%). Structural or functional cardiovascular anomalies were present in 17 fetuses (39%). The statistically significant correlation between the CVPS<8 and the poor outcome was confirmed (P<0.05). CONCLUSION: We recommend fetal echocardiography and CVPS evaluation before counseling in fetuses with cervicofacial tumors as cardiovascular dysfunction diagnosed prenatally may seriously affect survival of the fetus as well as prenatal counseling and need for cardiac treatment.

Cardiomyocyte Ogt is essential for postnatal viability.

The singly coded gene O-linked-ß-N-acetylglucosamine (O-GlcNAc) transferase (Ogt) resides on the X chromosome and is necessary for embryonic stem cell viability during embryogenesis. In mature cells, this enzyme catalyzes the posttranslational modification known as O-GlcNAc to various cellular proteins. Several groups, including our own, have shown that acute increases in protein O-GlcNAcylation are cardioprotective both in vitro and in vivo. Yet, little is known about how OGT affects cardiac function because total body knockout (KO) animals are not viable. Presently, we sought to establish the potential involvement of cardiomyocyte Ogt in cardiac maturation. Initially, we characterized a constitutive cardiomyocyte-specific (cm)OGT KO (c-cmOGT KO) mouse and found that only 12% of the c-cmOGT KO mice survived to weaning age (4 wk old); the surviving animals were smaller than their wild-type littermates, had dilated hearts, and showed overt signs of heart failure. Dysfunctional c-cmOGT KO hearts were more fibrotic, apoptotic, and hypertrophic. Several glycolytic genes were also upregulated; however, there were no gross changes in mitochondrial O2 consumption. Histopathology of the KO hearts indicated the potential involvement of endoplasmic reticulum stress, directing us to evaluate expression of 78-kDa glucose-regulated protein and protein disulfide isomerase, which were elevated. Additional groups of mice were subjected to inducible deletion of cmOGT, which did not produce overt dysfunction within the first couple of weeks of deletion. Yet, long-term loss (via inducible deletion) of cmOGT produced gradual and progressive cardiomyopathy. Thus, cardiomyocyte Ogt is necessary for maturation of the mammalian heart, and inducible deletion of cmOGT in the adult mouse produces progressive ventricular dysfunction.

[Antibodies to Hsp60 in newborn babies, to whom during the intervention for critical inborn heart failure, autologous cord blood was transfused, in early and late postoperative period].

The titre of antibodies to Hsp60 (heat shock protein) enhancement in the blood serum is considered a biological marker of poor state of organism. Comparative investigation was done on the antibodies titre to Hsp60 in the blood serum of a newborn babies, suffering critical inborn heart failure, to whom autologous cord blood or the donor's blood components was transfused, in early and remote postoperative period. In early postoperative period the lowering of the antibodies titre to Hsp60 in the blood serum was observed in comparison with them preoperatively, in a late period (in 2 yrs) in all the blood serum samples investigated antibodies to Hsp60 were not revealed. In 35% of patients, to whom the donor's blood components were transfused, there was registered the enhancement of the antibodies to Hsp60 titre in early postoperative period. High titre of antibodies have associated with enhanced rate of complications. In late postoperative period antibodies to Hsp60 were revealed in 20% of the examined patients.

Use of pimobendan in feline congenital heart failure.

A 6-month-old domestic shorthair cat was referred for evaluation of sudden lethargy and tachypnea following ovariohysterectomy. Upon failure of improvement with supportive care, a cardiologist identified congenital tricuspid dysplasia with signs of heart failure. Furosemide, enalapril, and pimobendan were used to reduce clinical signs and improve length and quality of life.

Usage de pimobendane lors d'une insuffisance cardiaque féline congénitale. Un chat commun domestique âgé de 6 mois a été recommandé pour l'évaluation d'un abattement et d'une tachypnée d'apparition soudaine après l'ovariohystérectomie. En absence d'amélioration avec des soins de soutien, un cardiologue a identifié une dysplasie tricuspidienne congénitale avec des signes d'insuffisance cardiaque. Le furosémide, l'enalapril et le pimobendane ont été utilisés pour atténuer les signes cliniques et améliorer la durée et la qualité de vie.(Traduit par Isabelle Vallières).

Empiric switch from calcineurin inhibitor to sirolimus-based immunosuppression in pediatric heart transplantation recipients.

Sirolimus is used in heart transplant patients with CAV and CNI-induced nephropathy. However, little is known regarding the tolerability, rejection rate, and effect on renal function when used empirically in children. We describe our experience with the empiric use of a sirolimus-based immunosuppressive regimen in pediatric heart transplantation recipients. We reviewed records of patients in whom conversion was attempted to a CNI-free sirolimus-based regimen. Rejection episodes and measures of renal function were recorded. We attempted to convert 20 patients, of which 16 were successful. In total, six of 20 patients (30%) experienced adverse effects. Of the 16 converted, four patients converted to sirolimus due to CNI-induced disease (three nephropathy, one CAV), while 12 patients (mean age 5.5 yr, range 0.1-21 yr; 33% female; 33% with a history of congenital heart disease) were empirically switched to sirolimus at a mean of 2.3 yr after transplant. Follow-up was available for a mean of 2.5 yr after conversion (range 0.5-8.3 yr). The rate of rejection while taking CNIs was 0.18 rejection episodes per patient-year (total of five episodes), compared with 0.03 rejection episodes per patient-year (total of one episode) while on sirolimus. Renal function, in terms of GFR, significantly improved after sirolimus conversion at latest follow-up (from 86 ± 37 mL/min to 130 ± 49 mL/min, p = 0.02). Here, we demonstrate the potential benefit of empiric use of sirolimus in pediatric heart transplant patients in a CNI-free regimen. Larger and longer studies are needed to further clarify risks of rejection and adverse effect profiles.

Prenatal intrarenal neuroblastoma mimicking a mesoblastic nephroma: a case report.

Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt.

Transcatheter embolization of congenital hepatic arteriovenous malformation using ethylene-vinyl alcohol copolymer (Onyx).

A male infant with high-output heart failure who had been found to have a hepatic arteriovenous malformation by ultrasound imaging was referred to our center (Department of Diagnostic and Interventional Radiology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Islamic Republic of Iran) for further evaluation. Computed tomography angiography revealed a large hepatic arteriovenous malformation with feeders originating from enlarged hepatic arteries and draining to enlarged hepatic veins. We performed a transcatheter embolization of the anomaly using ethylene vinyl alcohol copolymer (Onyx) during a single session. The cardiac function of the infant rapidly improved after the procedure. Over the 19 months of follow-up, his cardiac output remained stable and within the normal limits, and no complications were detected.

Right ventricular plication in a neonate with an unguarded tricuspid valvar orifice.

Unguarded tricuspid orifice is a congenital cardiac malformation characterized by absence of the tricuspid valvar leaflets in the setting of a normal atrioventricular junction and obligatory severe tricuspid regurgitation. A neonate with such a lesion presented with severe cardiac failure. The right atrium and ventricle were grossly dilated, and there were small muscular ventricular septal defects. We plicated the right-sided structures to create space for adequate expansion of the lungs and left ventricular filling, proceeding to staged conversion to the Fontan circulation. Our experience shows that neonates with cardiac failure due to dilated right-sided structures can be stabilized by surgical plication.

[Advances in pediatric cardiology and congenital heart diseases]. / Temas de actualidad en cardiología pediátrica y cardiopatías congénitas.

This article reviews the most significant developments reported between July 2006 and July 2007 in the fields of pediatric cardiology and congenital heart disease, in diagnosis and treatment in particular. In the area of imaging techniques, the increasing clinical roles of magnetic resonance imaging, tissue Doppler imaging and three-dimensional echocardiography are highlighted, as is the application of these techniques to fetal cardiology and its repercussions. The incidence and treatment of arrhythmias in children are also discussed and, with regard to heart failure, there is an assessment of recent findings in epidemiology and diagnosis, new drugs, ventricular assist systems, and the current status of heart transplantation. In the area of interventional cardiology, present-day techniques for the closure of atrial and ventricular septal defects are reviewed and there is an assessment of early results with partially absorbable devices, percutaneous pulmonary valve implantation, and new types of stent (e.g., premounted, coated and absorbable stents). In cardiac surgery, the focus is on studies that evaluate outcome quality, the search for new biocompatible conduits, experience with new techniques for treating complex transpositions (e.g., the Nikaidoh procedure and its variants), the medium- and long-term results of treating aortic valvular disease with pulmonary autografts (i.e., the Ross and Ross-Konno procedures), and current findings on the treatment of pulmonary insufficiency after correction of either the tetralogy of Fallot or a dysfunctional Fontan circuit.

Surgical correction of scoliosis in children with congenital heart failure (Fontan circulation): case report and literature review.

Patients with Fontan circulation represent a significant cardiorespiratory risk during spinal surgery. We report about two patients with severe scoliosis and Fontan circulation and their successful operative treatment. The case report will be compared with the national and international literature.