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Spectrum of cardiac lesions associated with Isolated Cleft Mitral Valve and their impact on therapeutic choices.

Arq Bras Cardiol. 2016 May;106(5):367-72

Authors: Hammiri AE, Drighil A, Benhaourech S

Abstract
BACKGROUND: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management.
METHODS: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014.
RESULTS: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation.
CONCLUSION: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.

PMID: 27096525 [PubMed - indexed for MEDLINE]

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Functional total anomalous pulmonary venous connection via levoatriocardinal vein.

Pediatr Int. 2016 Jul;58(7):656-9

Authors: Hayashi T, Ozawa K, Sugibayashi R, Wada S, Ono H

Abstract
We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the "dead-end" left atrium, the pulmonary venous flow had a to-and-fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left-side heart obstruction.

PMID: 27460400 [PubMed - indexed for MEDLINE]

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[Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome].

Rev Chil Pediatr. 2016 Sep - Oct;87(5):366-372

Authors: Becker Rencoret P, Besa Bandeira S, Riveros González S, Frangini Sanhueza P, Springmüller Pinto D, González Foretic R, Urcelay Montecinos G

Abstract
INTRODUCTION: During the last few years, numerous patients with univentricular heart disease have been treated surgically with total cavopulmonary anastomosis according to a staged surgery protocol in our institution.
OBJECTIVE: To evaluate the perioperative outcomes and survival of patients with hypoplastic left heart syndrome (HLHS) after the Fontan procedure and compare them with other types of univentricular heart disease.
PATIENTS AND METHOD: A total of 102 patients underwent a Fontan procedure between April 1996 and March 2014, 25 with HLHS (group I), and 77 patients with other types of univentricular heart disease (group II). Groups survival, demographics, hemodinamic studies, morbimortality, mechanical ventilation, surgical drains, post-operative stay, isotopes score, pacemaker use, and requiriment of Fontan takedown were analyzed.
RESULTS: Intraoperative mortality was 4% (n=1) for group I, and 7.8% (n=6) for group II (P=.451). A difference was only found in hospital length of stay (LOS), being 17 days (6-47) for group I and 12 days (5-103) for group II (P=.017). Mean follow-up was 4.24±2.08 years for group I, and 8.7±4.67 for group II. Survival rate at 8 years for both groups was 88%, and 81% at 10 years for group II.
CONCLUSIONS: The Fontan procedure had similar mortality, but longer LOS, in patients with HLHS compared to those with another types of single ventricle anatomy. Long term survival was comparable between both groups.

PMID: 27091396 [PubMed - indexed for MEDLINE]

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Diverse multi-organ histopathologic changes in a failed Fontan patient.

Pediatr Int. 2016 Oct;58(10):1061-1065

Authors: Mizuno M, Ohuchi H, Matsuyama TA, Miyazaki A, Ishibashi-Ueda H, Yamada O

Abstract
We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.

PMID: 27616284 [PubMed - indexed for MEDLINE]

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Clinically apparent long-term electric disturbances in the acute and very long-term of patent foramen ovale device-based closure.

Cardiovasc Revasc Med. 2017 Mar;18(2):118-122

Authors: Rigatelli G, Zuin M, Pedon L, Zecchel R, Dell'Avvocata F, Carrozza A, Zennaro M, Pastore G, Zanon F

Abstract
BACKGROUND/PURPOSE: Incidence of electrical disturbances in patients submitted to transcatheter patent foramen ovale (PFO) closure has not been fully clarified in a large population. The aim of the study is to assess the incidence of atrial fibrillation, supraventricular tachi-arrhythmias, and atrio-ventricular block in the acute and very long-term follow-up.
METHODS/MATERIALS: We reviewed the medical and instrumental data of 1000 consecutive patients (mean age 47.3±17.1years) prospectively enrolled in two centers over a 13-year period (February 1999 to February 2012) for right-to-left (R-to-L) shunt ICE-aided catheter-based closure using different devices.
RESULTS: Successful transcatheter PFO closure was achieved in 99.8% of the patients. Implanted devices were: Amplatzer PFO Occluder in 463 patients (46.3%), Amplatzer ASD Cribriform Occluder in 420 patients (42.0%), Premere Occlusion System in 95 patients (9.5%), and Biostar Occluder in 22 patients (2.2%). Postprocedural electrical complications occurred in 5.9% of patients. The only independent predictors of electrophysiological complications were female gender (OR 2.3, 0.5-5.1 [95% CI], p<0.001) and device disk >30mm (OR 5.0, 1.2-7.2 [95% CI], p<0.001). On a mean follow-up of 12 .3±0.6years (minimum 4- maximum 17years), electrical complications occurred in 1.4% of patients including one only case of complete AVB and 5 cases of permanent AF. The only independent predictors were female gender (OR 2.3, 0.5-5.1 [95% CI], p<0.001) and device disk >30mm (OR 5.0, 1.2-7.2 [95% CI], p<0.001).
CONCLUSION: Device-based closure of PFO using different devices, appeared very safe from an electrophysiological point of view with low incidence of electrical disturbances even in the very long-term follow-up.

PMID: 27847261 [PubMed - indexed for MEDLINE]

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Maternal and infant genetic variants, maternal periconceptional use of selective serotonin reuptake inhibitors, and risk of congenital heart defects in offspring: population based study.

BMJ. 2017 Mar 06;356:j832

Authors: Nembhard WN, Tang X, Hu Z, MacLeod S, Stowe Z, Webber D, National Birth Defects Prevention Study

Abstract
Objective To evaluate whether the association between maternal periconceptional use of selective serotonin reuptake inhibitors (SSRIs) and increased risk of congenital heart defects in offspring is modified by maternal or infant genetic variants in folate, homocysteine, or transsulfuration pathways.Design Population based study. DNA from mothers, fathers, and infants was genotyped with an Illumina GoldenGate custom single nucleotide polymorphism panel. A hybrid design based on a log linear model was used to calculate relative risks and Bayesian false discovery probabilities (BFDP) to identify polymorphisms associated with congenital heart defects modified by SSRI use.Data sources Data from the US National Birth Defects Prevention Study on 1180 liveborn infants with congenital heart defects and 1644 controls, born 1997-2008.Main outcome measures Cases included infants with selected congenital heart defects and control infants had no major defects. SSRI use was obtained from telephone interviews with mothers.Results For women who reported taking SSRIs periconceptionally, maternal SHMT1 (rs9909104) GG and AGgenotypes were associated with a 5.9 and 2.4 increased risk of select congenital heart defects in offspring, respectively, versus the AA genotype (BFDP=0.69). Compared with the AA genotype, BHMT (rs492842 and rs542852) GG and AG genotypes were associated with twice the riskof congenital heart defects (BFDP=0.74 and 0.79, respectively). MGST1 (rs2075237) CC and ACgenotypes were associated with an increased risk compared with the GG genotype (8.0 and 2.8, respectively; BFDP=0.79). Single nucleotide polymorphism in infant genes in the folate (MTHFS rs12438477), homocysteine (TRDMT1 rs6602178 and GNMT rs11752813) and transsulfuration (GSTP1 rs7941395 and MGST1 rs7294985) pathways were also associated with an increased risk of congenital heart defects.Conclusions Common maternal or infant genetic variants in folate, homocysteine, or transsulfuration pathways are associated with an increased risk of certain congenital heart defects among children of women taking SSRIs during cardiogenesis.

PMID: 28264803 [PubMed - indexed for MEDLINE]

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Coronary Artery Anomalies: When You Need to Worry.

Curr Cardiol Rep. 2017 May;19(5):39

Authors: Kochar A, Kiefer T

Abstract
PURPOSE OF REVIEW: There is a broad spectrum of coronary artery anomalies that cardiologists may encounter either incidentally or during evaluation for cardiac symptoms. These anomalies include anomalous coronary arteries arising from the opposite sinus of Valsalva (ACAOS), coronary fistulae, and coronary artery aneurysms. This manuscript outlines the unique features, diagnostic characteristics, and treatment considerations for these lesions.
RECENT FINDINGS: Intravenous ultrasound (IVUS), computed tomographic angiography (CTA), and magnetic resonance imaging (MRI) are becoming more sophisticated and will be increasingly used to facilitate the optimal treatment approach for coronary anomalies. There are a wide variety of coronary artery anomalies and their clinical ramifications range from benign to potentially fatal. Coronary anomalies often have complex anatomy and require advanced imaging modalities for comprehensive characterization. Due to the heterogeneity in lesion characteristics and outcomes, physicians should consider clinical and imaging features to create individualized management plans, along with referral to adult congenital heart disease centers.

PMID: 28374180 [PubMed - in process]

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Post mortem therapy from a subcutaneous ICD: What is the mechanism?

Pacing Clin Electrophysiol. 2017 Apr 04;:

Authors: Wiles BM, Fitzsimmons SJ, Roberts PR

PMID: 28374449 [PubMed - as supplied by publisher]

Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

Ann Am Thorac Soc. 2017 Apr 04;:

Authors: Mantegazza V, Apostolo A, Hager A

Abstract
Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries and new issues are emerging regarding them: the evaluation of their capacity to cope with physical activity, and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, pulmonary hypertension) and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak oxygen uptake, an early anaerobic threshold, a blunted heart rate response, a reduced rise of tidal volume, and an increased ratio of minute ventilation to carbon dioxide production. All these measures suggest common pathophysiological abnormalities 1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs or muscles, 2) chronotropic incompetence secondary to cardiac autonomic dysfunction or beta-blockers and antiarrhythmic therapy, and 3) ventilatory inefficiency caused by left-heart failure with pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease or cachexia. Most of these variables have also prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting life-style and therapeutic interventions.

PMID: 28375677 [PubMed - as supplied by publisher]

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Pre-pregnancy counseling for women with heart disease: A prospective study.

Int J Cardiol. 2017 Mar 30;:

Authors: Cauldwell M, Steer PJ, Swan L, Patel RR, Gatzoulis MA, Uebing A, Johnson MR

Abstract
BACKGROUND: Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received.
METHODS: Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders.
RESULTS: 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic.
CONCLUSIONS: There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

PMID: 28377190 [PubMed - as supplied by publisher]

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