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Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome.

ESC Heart Fail. 2020 Feb 20;:

Authors: Yamamoto M, Takashio S, Nakashima N, Hanatani S, Arima Y, Sakamoto K, Yamamoto E, Kaikita K, Aoki Y, Tsujita K

We present a case of double-chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation-associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not surgically treated, may cause paralysis of the extremities. We decided to pursue pharmacological therapy to control biventricular obstructions and reduce the perioperative complication rate. We initiated treatment with cibenzoline and bisoprolol; the doses were titrated according to the response. After 2 weeks, the peak pressure gradient of the two RV chambers decreased from 101 to 68 mmHg, and the LV peak pressure gradient decreased from 109 to 14 mmHg. Class 1A antiarrhythmic drugs and β-blockers decreased the severe pressure gradients of biventricular obstructions caused by DCRV and HOCM. The patient was able to undergo surgery to remove the intradural extramedullary tumour, which was diagnosed as schwannoma.

PMID: 32078254 [PubMed - as supplied by publisher]

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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

J Am Coll Cardiol. 2019 04 02;73(12):e81-e192

Authors: Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF

PMID: 30121239 [PubMed - indexed for MEDLINE]

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2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

J Am Coll Cardiol. 2019 04 02;73(12):1494-1563

Authors: Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF

PMID: 30121240 [PubMed - indexed for MEDLINE]

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Transaortic Flow in Aortic Stenosis: Stroke Volume Index versus Flow Rate.

Cardiology. 2018;141(1):71-73

Authors: Lazaros G, Drakopoulou MI, Tousoulis D

PMID: 30396182 [PubMed - indexed for MEDLINE]

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A case report of right coronary artery agenesis diagnosed by computed tomography coronary angiography.

Medicine (Baltimore). 2020 Feb;99(7):e19176

Authors: Forte E, Punzo B, Agrusta M, Salvatore M, Spidalieri G, Cavaliere C

INTRODUCTION: Single coronary artery is a rare condition characterized by the origin of a coronary that supplies the entire heart from a single coronary ostium.
PATIENT CONCERNS: A 45-year-old woman with an altered exercise testing was addressed to a computed tomography coronary angiography (CTCA) to rule out coronary artery disease (CAD).
DIAGNOSIS: CTCA examination showed the absence of the right coronary artery (RCA). The left anterior descending artery and the left circumflex artery (LCX) presented regular origin and course and LCX provided the posterior interventricular artery and the posterolateral artery.
INTERVENTIONS: As CTCA highlighted the absence of potentially life-threatening features related to coronary anomaly, no surgical treatment was advised.
OUTCOMES: The patient was dismissed, kept under pharmacological control and monitored over time.
CONCLUSION: CTCA is the first-choice imaging modality in patients with ECG abnormalities properly allowing the differential diagnosis between CAD and congenital heart disease.

PMID: 32049849 [PubMed - indexed for MEDLINE]

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A cell atlas of human thymic development defines T cell repertoire formation.

Science. 2020 Feb 21;367(6480):

Authors: Park JE, Botting RA, Domínguez Conde C, Popescu DM, Lavaert M, Kunz DJ, Goh I, Stephenson E, Ragazzini R, Tuck E, Wilbrey-Clark A, Roberts K, Kedlian VR, Ferdinand JR, He X, Webb S, Maunder D, Vandamme N, Mahbubani KT, Polanski K, Mamanova L, Bolt L, Crossland D, de Rita F, Fuller A, Filby A, Reynolds G, Dixon D, Saeb-Parsy K, Lisgo S, Henderson D, Vento-Tormo R, Bayraktar OA, Barker RA, Meyer KB, Saeys Y, Bonfanti P, Behjati S, Clatworthy MR, Taghon T, Haniffa M, Teichmann SA

The thymus provides a nurturing environment for the differentiation and selection of T cells, a process orchestrated by their interaction with multiple thymic cell types. We used single-cell RNA sequencing to create a cell census of the human thymus across the life span and to reconstruct T cell differentiation trajectories and T cell receptor (TCR) recombination kinetics. Using this approach, we identified and located in situ CD8αα+ T cell populations, thymic fibroblast subtypes, and activated dendritic cell states. In addition, we reveal a bias in TCR recombination and selection, which is attributed to genomic position and the kinetics of lineage commitment. Taken together, our data provide a comprehensive atlas of the human thymus across the life span with new insights into human T cell development.

PMID: 32079746 [PubMed - in process]

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Nationwide Frequency, Sequential Trends, and Impact of Co-morbid Mental Health Disorders on Hospitalizations, Outcomes, and Healthcare Resource Utilization in Adult Congenital Heart Disease.

Am J Cardiol. 2020 Jan 30;:

Authors: Desai R, Patel K, Dave H, Shah K, DeWitt N, Fong HK, Varma Y, Varma K, Mansuri Z, Sachdeva R, Khanna A, Kumar G

Despite the growing prevalence of adult congenital heart disease (ACHD), data on trends in prevalence of mental health disorders (MHD) among patients with ACHD remain limited. The National Inpatient Sample (2007 to 2014) was queried to identify the frequency and trends of MHD among ACHD hospitalizations (stratification by age, sex, and race); demographics and co-morbidities for ACHD cohorts, with (MHD+) versus without MHD (MHD-); the rate and trends of all-cause in-hospital mortality, disposition, mean length of stay, and hospitalization charges among both cohorts. A total of 11,709 (13.8%, mean age: 49.1 years, 56.0% females, 78.7% white) out of 85,029 ACHD patient encounters had a coexistent MHD (anxiety, depression, mood disorder, or psychosis). ACHD-MHD+ cohort was more often admitted nonelectively (38.1% vs 32.8%, p <0.001) and had a higher frequency of cardiac/extra-cardiac co-morbidities. The trends in prevalence of coexistent MHD increased from 10.3% to 17.5% (70% relative increase) from 2007 to 2014 with a consistently higher prevalence among females (from 13% to 20.3%) compared to males (from 7.6% to 15.5%) (ptrend <0.001). The hospitalization trends with MHD increased in whites (12.1% to 19.8%) and Hispanics (5.9% to 12.7%). All-cause mortality was lower (0.7% vs 1.1%, p = 0.002) in ACHD-MHD+; however, mean length of stay (∼5.7 vs 4.9 days, p <0.001) was higher without significant difference in charges ($97,710 vs $96,058, p = 0.137). ACHD-MHD+ cohort was less often discharged routinely (declining trend) and more frequently transferred to other facilities and required home healthcare (rising trends). In conclusion, this study reveals increasing trends of MHD, healthcare resource utilization and a higher frequency of co-morbidities in patients with ACHD.

PMID: 32085866 [PubMed - as supplied by publisher]

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Chemical Cardioversion of Atrial Flutter and Fibrillation in the Pediatric Population with Ibutilide.

Pacing Clin Electrophysiol. 2020 Feb 22;:

Authors: Dasgupta S, Dave I, Whitehill R, Fischbach P

INTRODUCTION: Atrial flutter (AFL) and atrial fibrillation (AF) are common in pediatric patients with congenital heart disease (CHD) and structurally normal hearts as well. Chemical cardioversion is attractive for patients with AFL/AF for a short period of time because of the ability to avoid sedation. We review a single center's experience with Ibutilide in pediatric patients in an effort to report on its safety and efficacy.
METHODS: We performed a retrospective chart review of pediatric patients (0-21 years) who underwent chemical cardioversion for AFL/AF with Ibutilide (January 2011-February 2019). Patients on another anti-arrhythmic medication or attempted chemical cardioversion with another drug were excluded.
RESULTS: There were 21 patients who met inclusion criteria. 13/21 (62%) patients were successfully cardioverted with ibutilide (10/13 had AF and 4/13 required a 2nd dose). There were no significant differences in baseline characteristics between those who were successfully cardioverted compared to those who were not. Administration of magnesium prior to administration did not appear to have an effect on the success rate. There was a significant increase in QTc intervals post Ibutilide administration which returned to baseline prior to discharge. One patient had symptomatic bradycardia needing intravenous fluids and another had torsades requiring electrical cardioversion during Ibutilide administration.
CONCLUSIONS: The success rate of chemical cardioversion with Ibutilide was similar in our experience as compared to studies in the adult population and the other lone pediatric study. While adverse events were uncommon, Ibutilide administration warrants close monitoring and fully defining its efficacy warrants further pediatric experience. This article is protected by copyright. All rights reserved.

PMID: 32086826 [PubMed - as supplied by publisher]

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Minimally Invasive Cardiac Surgery Without Peripheral Cannulation: A Single Centre Experience.

Heart Lung Circ. 2019 Nov;28(11):1728-1734

Authors: Kale SB, Ramalingam S

BACKGROUND: A minimally invasive approach without any peripheral cannulation was used as an alternative to median sternotomy for the correction of a wide range of cardiac defects with superior results and good cosmetic outcome.
METHODS: From October 2015 to March 2017, 145 patients underwent correction of congenital cardiac malformations with cardiopulmonary bypass through right anterior minithoracotomy (RAMT) with routine cannulation. The average age was 9.69±4.38years (range 2-21) and the average body surface area was 0.91±0.27square metres (range 0.5-1.7). The corrected defects included all types of atrial septal defect (ASD) and ventricular septal defects (VSD), partial atrioventricular septal defects with severe mitral regurgitation, mitral valve repair, repair of tetralogy physiology requiring outlet patch enhancement, isolated pulmonary stenosis (PS) and congenital coronary cameral fistula.
RESULTS: There was no operative mortality or major morbidity. All patients were alive at the time of this follow-up. The mean cardiopulmonary bypass time was 64.75±22.28minutes (range 30-175) and mean aortic cross clamp time was 37.53±18.23minutes (range 14-135). Fifty-five (55) patients were extubated in the operating room and the remaining 90 patients were extubated within 3hours in the intensive care unit. Repair and cosmetic results were excellent in all patients. There were no conversions to full sternotomy. No neurological events were reported.
CONCLUSIONS: Right anterior minithoracotomy without peripheral cannulation is safe and effective for the correction of a wide range of congenital heart defects including right ventricular outlet obstructions. The cosmetic results are satisfactory, avoiding psychosocial problems.

PMID: 30279108 [PubMed - indexed for MEDLINE]

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Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study.

J Thorac Cardiovasc Surg. 2019 04;157(4):1577-1587.e10

Authors: Jegatheeswaran A, Jacobs ML, Caldarone CA, Kirshbom PM, Williams WG, Blackstone EH, DeCampli WM, Duncan KF, Lambert LM, Walters HL, Tchervenkov CI, McCrindle BW

OBJECTIVES: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.
METHODS: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis.
RESULTS: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution.
CONCLUSIONS: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

PMID: 30770109 [PubMed - indexed for MEDLINE]