Journal Watch

French national survey on infective endocarditis and the Melody™ valve in percutaneous pulmonary valve implantation.

Arch Cardiovasc Dis. 2018 Mar 09;:

Authors: Malekzadeh-Milani S, Houeijeh A, Jalal Z, Hascoet S, Bakloul M, Aldebert P, Piéchaud JF, Heitz F, Bouvaist H, Dauphin C, Guérin P, Villemain O, Petit J, Godart F, Thambo JB, Boudjemline Y, French working group of Cardiac Catheterization in Congenital Heart Disease Patients

Abstract
BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a routine treatment for dysfunctional right ventricular outflow tract. Infective endocarditis (IE) is a major concern.
AIM: To report French experience with the Melody™ valve (Medtronic Inc., Minneapolis, MN, USA).
METHODS: All patients who underwent PPVI were recorded in a multicentre French national survey. Demographic and procedural data were collected from patients with IE. Bacterial identification, diagnostic tools and outcome were recorded.
RESULTS: Forty-five cases of IE were diagnosed in 43 patients. The cumulative IE incidence was 11.8% (95% confidence interval [CI] 8.5-15.9). The annualized IE incidence was 3.6% (95% CI 0-4.8). Freedom from IE was 96.3% and 85.8% at 12 months and 60 months, respectively. IE incidence did not change during the study period. The mean interval between PPVI and IE was 2.6±2.1 years (range, 5 days to 7.3 years). Fifteen patients with IE required intravenous antibiotics only. Seven patients had early interventional cardiac catheterization to relieve severe right ventricular outflow tract obstruction. Twenty-four patients had surgical valve replacement (six urgently; nine semi-urgently; nine electively). Staphylococcus aureus IE required surgery in all but one patient. Three patients died before any treatment. Three additional patients died, giving a mortality rate of 14%. Global survival in the total cohort of patients who received a Melody valve was excellent (96.5% at 5 years). When comparing survival curves between the IE and non-IE groups, death and cardiovascular events were statistically significantly higher in the IE group (log-rank P<0.0001).
CONCLUSION: Melody valve IE is a severe complication following PPVI. The annualized IE incidence in this cohort was similar to rates reported in other studies. With rapid diagnosis and adequate treatment, outcome has improved, and unfavourable outcome is mainly associated with S. aureus.

PMID: 29530718 [PubMed - as supplied by publisher]

Physiology of Cardiac Development: From Genetics to Signaling to Therapeutic Strategies.

Curr Opin Physiol. 2018 Feb;1:123-139

Authors: Sun C, Kontaridis MI

Abstract
The heart is one of the first organs to form and function during embryonic development. It is comprised of multiple cell lineages, each integral for proper cardiac development, and include cardiomyocytes, endothelial cells, epicardial cells and neural crest cells. The molecular mechanisms regulating cardiac development and morphogenesis are dependent on signaling crosstalk between multiple lineages through paracrine interactions, cell-ECM interactions, and cell-cell interactions, which together, help facilitate survival, growth, proliferation, differentiation and migration of cardiac tissue. Aberrant regulation of any of these processes can induce developmental disorders and pathological phenotypes. Here, we will discuss each of these processes, the genetic factors that contribute to each step of cardiac development, as well as the current and future therapeutic targets and mechanisms of heart development and disease. Understanding the complex interactions that regulate cardiac development, proliferation and differentiation is not only vital to understanding the causes of congenital heart defects, but to also finding new therapeutics that can treat both pediatric and adult cardiac disease in the near future.

PMID: 29532042 [PubMed]

Related Articles

Four successful pregnancies in a patient with Fontan palliation and congenital heart disease: a case report.

J Cardiothorac Surg. 2017 Nov 28;12(1):104

Authors: Al Najashi KS, Mehdi S, Mohsin S, Atiyah M, Abdullah HA, Abdulhameed J, Al Zahrani AM

Abstract
BACKGROUND: Fontan is a palliative procedure in patients with single ventricle. Single ventricle supports systemic cardiac output and pulmonary blood flow is passively directed to the right pulmonary artery. Women with Fontan palliation are reported to have increased maternal risk during pregnancy. There are few reports of successful pregnancies in such cases. However data on these pregnancies is lacking, we consider this to be the first reported from kingdom of Saudi Arabia.
CASE PRESENTATION: We present a 35-year-old woman from the Kingdom of Saudi Arabia who had Fontan surgery and who had four successful pregnancies and multiple miscarriages. She delivered live, low birth weight neonates.
CONCLUSION: This report provides an anecdotal evidence that pregnancy can be tolerated in an adequate Fontan patient with successful outcomes.

PMID: 29183331 [PubMed - indexed for MEDLINE]

Current state of risk stratification for sudden cardiac death in adults with congenital heart disease.

Anatol J Cardiol. 2018 Mar 13;:

Authors: Vehmeijer JT, Mulder BJ, de Groot JR

Abstract
Sudden cardiac death (SCD), mainly caused by ventricular arrhythmias, is one of the leading causes of mortality in adult congenital heart disease (ACHD) patients. An implantable cardioverter defibrillator (ICD) may prevent SCD, but risk stratification remains challenging. In this review, we will address the current guideline recommendations for ICD implantation in ACHD patients, as well as review a recent study in which the discriminative ability for SCD of these guidelines is evaluated. In this study, the guideline recommendations were applied to patients who died of SCD and living controls. Among SCD cases, 35%-41% of patients were recommended ICD, whereas 16%-17% of controls were recommended ICD. The discriminative ability for SCD of the guidelines was poor, with an area under the receiver operating characteristic curve of 0.61-0.63. Risk stratification for SCD in ACHD patients, therefore, remains to be a work-in-progress.

PMID: 29537971 [PubMed - as supplied by publisher]

Association of Albuminuria With Major Adverse Outcomes in Adults With Congenital Heart Disease: Results From the Boston Adult Congenital Heart Biobank.

JAMA Cardiol. 2018 Mar 14;:

Authors: Rajpal S, Alshawabkeh L, Almaddah N, Joyce CM, Shafer K, Gurvitz M, Waikar SS, Mc Causland FR, Landzberg MJ, Opotowsky AR

Abstract
Importance: Albuminuria is associated with adverse outcomes in diverse groups of patients, but the importance of albuminuria in the emerging population of increasingly complex adults with congenital heart disease (ACHD) remains unknown.
Objective: To assess the prevalence, risk factors, and prognostic implications of albuminuria in ACHD.
Design, Setting, and Participants: This prospective study assessed a cohort of ambulatory patients aged 18 years and older who were examined at an ACHD referral center and enrolled in the Boston ACHD Biobank between May 17, 2012, to August 5, 2016. Albuminuria was defined as an urine albumin-to-creatinine (ACR) ratio of 30 mg/g or more.
Main Outcomes and Measures: Death or nonelective cardiovascular hospitalization, defined as overnight admission for heart failure, arrhythmia, thromboembolic events, cerebral hemorrhage, and/or disease-specific events.
Results: We measured the ACR of 612 adult patients with CHD (mean [SD] age, 38.6 [13.4] years; 308 [50.3%] women). Albuminuria was present in 106 people (17.3%) and was associated with older age (patients with ACR <30 mg/g: mean [SD]: 37.5 [13.2] years; vs patients with ACR ≥30 mg/g: 43.8 [13.1] years; P < .001), presence of diabetes mellitus (ACR <30 mg/g: 13 of 506 [2.6%]; vs ≥30 mg/g: 11 of 106 [10.4%]; P < .001), lower estimated glomerular filtration rate (ACR <30 mg/g: median [interquartile range (IQR)]: 103.3 [90.0-116.4] mL/min/1.73 m2; ACR ≥30 mg/g: 99.1 [78.8-108.7] mL/min/1.73 m2; P = .002), and cyanosis (ACR <30 mg/g: 23 of 506 [5.1%]; vs ACR ≥30 mg/g: 21 of 106 [22.6%]; P < .001). After a mean (SD) follow-up time of 270 (288) days, 17 patients (2.5%) died, while 68 (11.1%) either died or experienced overnight inpatient admission. Albuminuria predicted outcome, with 30 of 106 patients with albuminuria (28.3%) affected vs 38 of 506 patients without albuminuria (7.5%; hazard ratio [HR], 3.0; 95% CI, 1.9-4.9; P < .001). Albuminuria was also associated with increased mortality (11 of 106 [10.4%]; vs 6 of 506 [1.2%] in patients with and without albuminuria, respectively; HR, 6.4; 95% CI, 2.4-17.3; P < .001). Albuminuria was associated with the outcomes only in patients with a biventricular circulation (HR, 4.5; 95% CI, 2.5-8.0) and not those with single-ventricle circulation (HR, 1.0; 95% CI, 0.4-2.8; P = 0.01 compared with biventricular circulation group). Among 133 patients (21.7%) in NYHA functional class 2, albuminuria was strongly associated with death or nonelective hospitalization.
Conclusions and Relevance: Albuminuria is common and is associated with increased risk for adverse outcome in patients with ACHD with biventricular circulation. Albuminuria appears especially useful in stratifying risk in patients categorized as NYHA functional class 2.

PMID: 29541749 [PubMed - as supplied by publisher]

Functional classification of heart failure before and after implementing a healthcare transition program for youth and young adults transferring from a pediatric to an adult congenital heart disease clinics.

Congenit Heart Dis. 2018 Mar 15;:

Authors: Hergenroeder AC, Moodie DS, Penny DJ, Wiemann CM, Sanchez-Fournier B, Moore LK, Head J

Abstract
OBJECTIVE: To describe changes in functional status between the last pediatric and first adult congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after implementing a healthcare transition (HCT) planning program.
DESIGN: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult CHD Clinic before the intervention.
SETTING: Texas Children's Hospital (TCH).
PATIENTS: Sixteen to 25-year-olds, cognitively normal, English speaking patients with moderate to severe CHD who transitioned from the Pediatric to the Adult CHD clinic.
INTERVENTIONS: An EMR-based transition planning tool (TPT) was introduced into the Pediatric CHD Clinic. Two nurses used the TPT with eligible patients. Independent of the intervention, two medicine-pediatric CHD physicians and one nurse practitioner were added to the ACHD Clinic to address growing capacity needs.
OUTCOME MEASURES: The New York Heart Association Functional Classification of Heart Failure (NYHAFS).
RESULTS: Control patients waited 26 ± 19.2 months after their last pediatric clinic visit for their first adult visit. Intervention patients waited 13 ± 8.3 months (P = .019). Control and Intervention patients experienced a lapse in care greater than two (50% vs 13%, P = .017) and three (30% vs 0%, P = .011) years, respectively. The difference between the recommended number of months for follow-up and the first adult appointment (15.1 ± 17.3 Control and 4.4 ± 6.1 Intervention months) was significant (P = .025). NYHAFS deteriorated between the last Pediatric visit and the first ACHD visit for seven (23%) Control patients and no Intervention patients (P = .042). Four of seven Control patients whose NYHAFS declined had a lapse of care of more than two years.
CONCLUSIONS: There is a need for improved HCT planning for patients with moderate to severe CHD, otherwise, lapses of care and adverse outcomes can ensue.

PMID: 29542247 [PubMed - as supplied by publisher]

Anesthesia for Percutaneous Pulmonary Valve Implantation: A Case Series.

Anesth Analg. 2018 Mar 14;:

Authors: Arboleda Salazar R, Heggie J, Wolski P, Horlick E, Osten M, Meineri M

Abstract
BACKGROUND: Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center.
METHODS: The study consisted of a chart review of patients who underwent TPVR at Toronto General Hospital between 2006 and 2015. Information about preoperative assessment, intraoperative anesthetic management, and intra- and postprocedural complications was collected. Two types of percutaneous valves have been used for a conduit or valve size between 16 and 28 mm. These procedures are done via the femoral, jugular, or subclavian vein under general anesthesia.
RESULTS: Seventy-nine adults (17-68 years of age) who underwent elective TPVR procedures were included. General anesthesia was used in all cases. Defibrillation was necessary in 1 case, and bradycardia was spontaneously resolved in another 1. Eighty-five percent were successfully extubated at the end of the procedure. Five patients required intraoperative inotropic support. Three patients presented self-resolved hemoptysis. Mechanical ventilation for >24 hours was necessary in 3 cases, 2 of which also required concomitant inotropic support. Four failed deployments and 1 case of persistent conduit stenosis were reported. Three patients required reintubation. All patients were discharged home.
CONCLUSIONS: Patients undergoing TPVR represent a complex and heterogeneous population. General anesthesia with endotracheal intubation is preferred. Setup for urgent lung isolation and cardiac defibrillation should be considered. Postoperative monitoring and intensive care setting are required. Anesthesiologists with cardiac anesthesia training are probably better suited to manage these patients.

PMID: 29543640 [PubMed - as supplied by publisher]

A Randomized Trial Comparing Cardiac Rehabilitation to Standard of Care for Adults With Congenital Heart Disease.

World J Pediatr Congenit Heart Surg. 2018 Mar;9(2):185-193

Authors: Opotowsky AR, Rhodes J, Landzberg MJ, Bhatt AB, Shafer KM, Yeh DD, Crouter SE, Ubeda Tikkanen A

Abstract
BACKGROUND: Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD).
METHODS: We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O2 saturation ≥92%, and had peak O2 consumption ([Formula: see text]) < 80% predicted. We assessed exercise capacity, physical activity, quality of life, self-reported health status, and other variables at baseline and after 12 weeks. The prespecified primary end point was change in [Formula: see text].
RESULTS: We analyzed data on 28 participants (aged 41.1 ± 12.1 years, 50% male), 13 randomized to CR and 15 to SOC. [Formula: see text] averaged 16.8 ± 3.8 mL/kg/min, peak work rate = 95 ± 28 W, and median Minnesota Living with Heart Failure Questionnaire (MLHFQ) score = 27 (interquartile range: 11-44). Cardiac rehabilitation participants were older (48 ± 9 years vs 36 ± 12 years; P = .01), but there were no significant between-group differences in other variables. There were no adverse events related to CR. [Formula: see text] increased in the CR group compared with SOC (+2.2 mL/kg/min, 95% confidence interval: 0.7-3.7; P = .002, age-adjusted +2.7 mL/kg/min; P = .004); there was a nonsignificant improvement in work rate (+8.1 W; P = .13). Among the 25 participants with baseline MLHFQ > 5, there was a clinically important >5-point improvement in 72.7% and 28.6% of CR and SOC participants, respectively ( P = .047). Cardiac rehabilitation was also associated with improved self-assessment of overall health ( P < .04).
CONCLUSIONS: Cardiac rehabilitation is safe and is associated with improvement in aerobic capacity and self-reported health status compared with SOC in adults with CHD.

PMID: 29544423 [PubMed - in process]

Too big for echocardiography.

Eur Heart J. 2018 Mar 12;:

Authors: Karsenty C, Ladouceur M, Laredo M, Iserin L, Soulat G

PMID: 29546363 [PubMed - as supplied by publisher]

Transcatheter Pulmonary Valve Replacement: Current State of Art.

Curr Cardiol Rep. 2018 Mar 15;20(4):27

Authors: Alkashkari W, Alsubei A, Hijazi ZM

Abstract
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction. This review aims to summarize (1) the current state of the art for tPVR, (2) the expanding indications, and (3) the technological obstacles to optimizing tPVR.
RECENT FINDINGS: Since its introduction in 2000, more than ten thousands tPVR procedures have been performed worldwide. Although the indications for tPVR have been adapted earlier from those accepted for surgical intervention, they remain incompletely defined. The new imaging modalities give better assessment of cardiac anatomy and function and determine candidacy for the procedure. The procedure has been shown to be feasible and safe when performed in patients who received pulmonary conduit and or bioprosthetic valves between the right ventricle and the pulmonary artery. Fewer selected patients post trans-annular patch repair for tetralogy of Fallot may also be candidates for this technology. Size restrictions of the currently available valves limit deployment in the majority of patients post trans-annular patch repair. Newer valves and techniques are being developed that may help such patients. Refinements and further developments of this procedure hold promise for the extension of this technology to other patient populations.

PMID: 29546472 [PubMed - in process]

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