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Fetal and Postnatal Echocardiographic Diagnosis of Ebstein Anomaly of the Mitral Valve.

Pediatr Cardiol. 2018 Aug;39(6):1276-1279

Authors: Aly S, Bokowski J, Diab K, Muller BA

Abstract
Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement into LV cavity. Postnatal transthoracic and transesophageal echocardiograms confirmed the diagnosis with apical displacement of the level of coaptation MV into the LV cavity. To the best of our knowledge, fetal diagnosis of Ebstein anomaly of MV has not yet been reported in the medical literature.

PMID: 29756160 [PubMed - indexed for MEDLINE]

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Successful simultaneous transcatheter treatment for a secundum atrial septal defect complicated by valvular pulmonary stenosis in an infant.

Cardiol Young. 2018 Oct;28(10):1162-1164

Authors: Asada D, Tomita H, Fujii T

Abstract
Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully. Simultaneous transcatheter correction is an effective therapeutic option even in infants.

PMID: 29991370 [PubMed - indexed for MEDLINE]

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Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

Int J Cardiol. 2018 Oct 26;:

Authors: Troost E, Roggen L, Goossens E, Moons P, De Meester P, Van De Bruaene A, Budts W

Abstract
As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.

PMID: 30413300 [PubMed - as supplied by publisher]

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Assessment of a novel radiation reduction protocol for pediatric and adult congenital device implantation.

J Interv Card Electrophysiol. 2018 Nov 09;:

Authors: Clark BC, Janson CM, Ceresnak SR, Osei FA, Bonney WJ, Nappo L, Pass RH

Abstract
PURPOSE: Device implantation requires fluoroscopic guidance, which carries inherent risks of ionizing radiation. We evaluated the impact of a low-dose fluoroscopic protocol on radiation exposure during device implantation.
METHODS: All patients who underwent pacemaker or ICD implantation with new transvenous leads from July 2011 to January 2018 were included. A novel ALARA protocol consisting of ultra-low frame rates (2-3 frames/s), low dose/frame (6-18 mGy/frame), and use of the "air-gap" technique in patients < 20 kg was employed. Demographics, procedural data, and radiation exposure levels were collected and analyzed.
RESULTS: Thirty patients underwent device implantation without additional catheterization, electrophysiology study, or ablation procedure (median age 15 years; range 5-50) with a total of 43 leads placed. Forty-seven percent of patients had a primary rhythm disturbance, 33% had cardiomyopathy, and 20% had congenital heart disease. Fifty percent were pacemakers (53% dual-chamber, 27% ventricle, 20% atrial) and 50% of devices implanted were ICDs (87% single-chamber). All implants were acutely successful with acceptable atrial and ventricular sensing and capture thresholds. The median fluoroscopy time was 11.5 min (inter-quartile range (IQR) 8.0-18.2), median air kerma dose 4.0 mGy (IQR 2.5-19.5), and median dose-area product 27.8 μGy/m2 (IQR 17.1-106.5). Median implant procedure time was 133 min. One patient required revision secondary to device migration without lead derangement 2 days post-procedure.
CONCLUSIONS: Use of a novel fluoroscopic protocol may help decrease radiation exposure to patients and staff without affecting efficacy or risk. These data may represent benchmarks against which future device implantation procedures can be compared.

PMID: 30413993 [PubMed - as supplied by publisher]

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Current understanding and perioperative management of pediatric pulmonary hypertension.

Paediatr Anaesth. 2018 Nov 10;:

Authors: Latham GJ, Yung D

Abstract
Pediatric pulmonary hypertension (PH) is a complex disease with multiple, diverse etiologies affecting the premature neonate to the young adult. Pediatric pulmonary arterial hypertension (PAH), whether idiopathic or associated with congenital heart disease, is the most commonly discussed form of pediatric PH, as it is progressive and lethal. However, neonatal forms of PH are vastly more frequent, and while most cases are transient, the risk of morbidity and mortality in this group deserves recognition. PH due to left heart disease is another subset increasingly recognized as an important cause of pediatric PH. One aspect of pediatric PH is very clear: anesthetizing the child with PH is associated with a significantly heightened risk of morbidity and mortality. It is therefore imperative that anesthesiologists who care for children with PH have a firm understanding of the pathophysiology of the various forms of pediatric PH, the impact of anesthesia and sedation in the setting of PH, and anesthesiologists' role as perioperative experts from preoperative planning to postoperative disposition. This review summarizes the current understanding of pediatric PH physiology, preoperative risk stratification, anesthetic risk, and intraoperative considerations relevant to the underlying pathophysiology of various forms of pediatric PH. This article is protected by copyright. All rights reserved.

PMID: 30414333 [PubMed - as supplied by publisher]

Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease.

Int J Cardiol. 2018 Oct 27;:

Authors: Vehmeijer JT, Hulleman M, Kuijpers JM, Blom MT, Tan HL, Mulder BJM, de Groot JR, Koster RW

Abstract
AIMS: Adult congenital heart disease (ACHD) patients are at increased risk of sudden cardiac death and out-of-hospital cardiac arrest (OHCA). Currently, insufficient data exist on outcome, causes and circumstances of OHCA of ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population.
METHODS AND RESULTS: We identified ACHD patients with OHCA by linking data from a Dutch nationwide registry of ACHD patients (CONCOR, n = 15,727), and ARREST, a cohort of OHCA cases (n = 17,868). 62 ACHD patients with OHCA were identified. Ventricular septal defect (n = 11), bicuspid aortic valve (n = 10) and atrial septal defect (n = 8) were the most common diagnoses. We included OHCA cases from the general population as controls. ACHD patients were younger than controls (n = 11,624) at the time of OHCA (47 (SD ± 17) years vs. 66 (SD ± 15) years, respectively, p < 0.001), and more often had a shockable initial rhythm (67% vs 40%, respectively, p < 0.001). A cardiac cause of OHCA was identified in 76% of ACHD patients, with only 7% due to myocardial infarction or ischemia. Survival was better in ACHD patients than in controls (44% vs. 19%, p < 0.001), but this difference disappeared after correction for age, gender, witnessed arrest, bystander resuscitation, public location and shockable rhythm.
CONCLUSIONS: OHCA in ACHD patients occurs at young age, is rarely caused by ischemia and occurs mainly in patients with simple congenital defects. Risk stratification efforts should therefore not be restricted to ACHD patients with severe congenital defects.

PMID: 30414749 [PubMed - as supplied by publisher]

Long-term outcome after atrial correction for transposition of the great arteries.

Heart. 2018 Nov 10;:

Authors: Couperus LE, Vliegen HW, Zandstra TE, Kiès P, Jongbloed MRM, Holman ER, Zeppenfeld K, Hazekamp MG, Schalij MJ, Scherptong RWC

Abstract
OBJECTIVE: This study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA).
METHODS: In 76 adult patients with TGA (59% male) after atrial correction, long-term survival and morbidity were investigated in three periods: early (<15 years postoperatively), midterm (15-30 years postoperatively) and late (>30 years postoperatively).
RESULTS: The Mustard technique was performed in 41 (54%) patients, and the Senning technique was performed in 35 (46%) patients aged 3.1 (IQR: 2.1-3.8) and 1.0 (IQR: 0.6-3.1; p<0.01) years, respectively. Adult survival was 82% at 39.7 (IQR: 35.9-42.4) years postoperatively and exceeded 50 years in four patients. Supraventricular tachycardia (SVT) occurred in 51% of patients. The incidences of ventricular arrhythmia (0%, 8% and 13%; p<0.01), heart failure (0%, 5% and 19%; p<0.01) and surgical reinterventions (0%, 5% and 11%; p=0.01) increased from early to late follow-up. At last follow-up, RV function was depressed in 31 (46%) patients, and New York Heart Association functional class was ≥2 in 34 (48%) patients. Bradyarrhythmia, SVT and ventricular arrhythmia were associated with depressed RV function (OR: 4.47, 95% CI 1.50 to 13.28, p<0.01; OR: 3.74, 95% CI 1.26 to 11.14, p=0.02; OR: 14.40, 95% CI 2.80 to 74.07, p<0.01, respectively) and worse functional capacity (OR: 2.10, 95% CI 0.75 to 5.82, p=0.16; OR: 2.87, 95% CI 1.06 to 7.81, p=0.04; OR: 8.47, 95% CI 1.70 to 42.10, p<0.01, respectively).
CONCLUSIONS: In adult patients with TGA, survival was 82% at 39.7 (IQR: 35.9-42.4) years after atrial correction. Morbidity was high and included SVT as most frequent adverse event. Ventricular arrhythmias, heart failure and surgical reinterventions were common during late follow-up. Adverse events were associated with depressed right ventricle function and reduced functional class.

PMID: 30415204 [PubMed - as supplied by publisher]

Single-center experience of hemodialysis in patients after Fontan palliation.

Int J Cardiol Heart Vasc. 2018 Dec;21:94-95

Authors: Young B, Franklin W, Lam W, Ermis P

PMID: 30417073 [PubMed]

Implantation of the Melody transcatheter pulmonary valve PB1016 in patients with dysfunctional right ventricular outflow tract conduits.

Catheter Cardiovasc Interv. 2018 Nov 12;:

Authors: Morray BH, Jones TK, Coe JY, Gitter R, Martinez JZ, Turner DR, Gray RG, Lung TH, Berman DP, Levi DS

Abstract
OBJECTIVES: This study describes procedural and 1-year outcomes of the 16 mm Melody PB1016 valve in patients with dysfunctional RVOT conduits.
BACKGROUND: The Melody PB1016 is a standard Melody valve produced from a 16 mm bovine jugular vein and is intended for deployment up to 20 mm.
METHODS: This is a prospective, non-randomized, multicenter study of the procedural and short-term outcomes of Melody PB1016 TPV replacement within dysfunctional RVOT conduits. Data from eight centers were included in the analysis.
RESULTS: During the study period, 39 patients underwent attempted Melody TPVR. Of the 39 patients, 30 underwent successful Melody TPVR. The majority of patients underwent placement of one or more stents prior to TPVR. There was a significant reduction in peak conduit pressure gradient following TPVR (38 mmHg vs. 11 mmHg, P < 0.001). There were three cases of confined conduit tears successfully treated with covered stents or the valve itself. Repeat catheterization was performed in one patient for early re-obstruction that was successfully treated with balloon valvuloplasty. At recent follow-up, there were no cases of more than mild valve regurgitation and the mean pulmonary valve gradient by echocardiogram remained reduced relative to pre-TPVR implant measurements (33.5 mmHg vs. 15.2 mmHg). There were no cases of valve stent fracture or endocarditis reported at the 1-year follow-up.
CONCLUSIONS: Our analysis of TPVR with the PB1016 valve in RVOT conduits showed it to be safe and effective and can be performed in a wide range of conduit sizes with preserved valve function. ClinicalTrials.gov Identifier: NCT02347189.

PMID: 30419603 [PubMed - as supplied by publisher]

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Postcardiotomy refractory ventricular fibrillation: rescue using veno-arterial extracorporeal membrane oxygenation.

Perfusion. 2018 07;33(5):401-403

Authors: Soynov I, Kornilov I, Zubritskiy A, Ponomarev D, Nichay N, Gorbatykh A, Voitov A, Karaskov A

Abstract
We present a case of 7-hour ventricular fibrillation with successful use of veno-arterial extracorporeal membrane oxygenation as a bridge to recovery in a 30-year-old patient with grown-up congenital heart disease who underwent pulmonary valve replacement.

PMID: 29228895 [PubMed - indexed for MEDLINE]

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