The Pulse – May 2019

The Pulse is a summary of relevant news and articles for Congenital Heart Professionals and highlights:

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What's Been Happening?

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Long-term outcomes of cardiac resynchronization therapy in adult congenital heart disease.

Leyva F, Zegard A, Qiu T, de Bono J, Thorne S, Clift P, Marshall H, Hudsmith L.

Pacing Clin Electrophysiol. 2019 Mar 25. doi: 10.1111/pace.13670. [Epub ahead of print]

PMID: 30908673

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Take Home Points: from Dr. Jeremy Moore (Los Angeles)


·    ACHD patients undergoing CRT appear to fare reasonably well at mid-term follow up.

·    In comparison with patients affected by ischemic and non-ischemic cardiomyopathy, ACHD patients undergoing CRT experience similar cardiovascular mortality and need for heart failure admission.

·    When adjusted for clinically-relevant covariates, ACHD patients demonstrate similar mortality after CRT as their non-ACHD counterparts.


Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction.

White BR, Ho DY, Faerber JA, Katcoff H, Glatz AC, Mascio CE, Stephens P Jr, Cohen MS.

Ann Thorac Surg. 2019 Mar 15. pii: S0003-4975(19)30354-6. doi: 10.1016/j.athoracsur.2019.02.017. [Epub ahead of print]

PMID: 30885849

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Take Home Points: from Dr. Timothy Pirolli (Dallas)


·    Patients born with total anomalous pulmonary venous return have a high risk of recurrent obstruction post-repair though there is still a question of which patients are at highest risk.

·    This retrospective, single-center study evaluated their experience with TAPVR repair and sought to identify risk factors for postoperative pulmonary venous obstruction as well as to try to standardize (and risk stratify) the preoperative echo findings for postoperative obstruction.


Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.   

Padalino MA, Franchetti N, Hazekamp M, Sojak V, Carrel T, Frigiola A, Lo Rito M, Horer J, Roussin R, Cleuziou J, Meyns B, Fragata J, Telles H, Polimenakos AC, Francois K, Veshti A, Salminen J, Rocafort AG, Nosal M, Vedovelli L, Guariento A, Vida VL, Sarris GE, Boccuzzo G, Stellin G.

Eur J Cardiothorac Surg. 2019 Mar 20. pii: ezz080. doi: 10.1093/ejcts/ezz080. [Epub ahead of print]

PMID: 30897195

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Take-Home Points: from Dr. Michael Ma (Stanford, CA)


·    AAOCA can be clinically very significant, resulting in sudden cardiac death and/or acute profound ventricular dysfunction. The management of clinically significant AAOCA involves surgical correction by a number of techniques that are generally very safe, with little morbidity and mortality.

·    In the largest surgical AAOCA series published to date, operative and long-term mortality was 1.3% and 2.2% respectively. Event-free survival was estimated to 74.6% at 20-25 year follow-up. Most patients remained in excellent functional status, and a small subset continued to have poorly-defined chest pain after surgical repair.


The long-term functional outcome in Mustard patients study: Another decade of follow-up.

Srivastava NT, Hurwitz R, Kay WA, Eckert GJ, Kuhlenhoelter A, DeGrave N, Ebenroth ES.

Congenit Heart Dis. 2019 Mar;14(2):176-184. doi: 10.1111/chd.12698. Epub 2018 Nov 23.

PMID: 30468301

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Take Home Points: from Dr. Timothy Roberts (Melbourne)


·    Cardiac mortality (death or transplant) is high in D-TGA patients treated with the Mustard atrial switch procedure, followed longitudinally over several decades, with half of the events occurring after age 25.

·    The majority of deaths were due to systemic RV failure rather than sudden cardiac death.

·    3/8 patients with sudden cardiac death (excluding those post-transplant) had no prior history of arrhythmia during routine follow up.

·    Systemic RV function showed a progressive decline over longitudinal follow up, whereas sub-pulmonary LV function was maintained.

·    The majority of patients demonstrated some degree of arrhythmia.

·    Few patients required pacemaker due to sinus node dysfunction/sick sinus syndrome beyond 2-3 decades post-surgery.

·    General health, physical function and energy were significantly reduced in quality of life questionnaires.

·    There was no single clear predictor for worse outcomes in D-TGA Mustard patients, although deteriorating RVEF is associated with mortality and transplantation.



Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.

Lee MGY, Babu-Narayan SV, Kempny A, Uebing A, Montanaro C, Shore DF, d’Udekem Y, Gatzoulis MA.

Heart. 2019 Mar 28. pii: heartjnl-2018-314257. doi: 10.1136/heartjnl-2018-314257. [Epub ahead of print]

PMID: 30923175

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Take Home Points: from Dr. Damien Cullington (Leeds to Liverpool)


·    Large retrospective, single-centre analysis of the contemporary outcomes of patients following coarctation (CoA) repair.

·    Initial cohort comprised 834 patients aged > 16 years old.

·    The median age of repair was 4 yrs. old (95% CI 1 month-15 years old).

·    In 83% (n=690) repair was surgical and the remainder, percutaneously.

·    60% of surgical repairs were performed pre 1990 and 58% were end-to end anastomotic type.

·    Median follow up 27 years. Deaths occurring >30 days (‘late’) after CoA repair occurred in 5% of patients (n=38).

·    Almost a third (n=246) needed > an aortic arch intervention (n=234); 111 required an aortic valve intervention and 43 needed an ascending aortic intervention.

·    In 59% of patients, (n=284/474) a residual pressure drop (> 25mmHg) or repair site/diaphragm ratio < 70% was seen at latest follow up.

·    A high burden of hypertension in the cohort – 70% of patients with available data were hypertensive (n=379/661) or prehypertensive (83/661).

·    Survival in patients with a history of CoA was 99%, 88% and 65% at 30, 50 and 70 years of age – the standardised mortality ratio was 3.20 compared to age/sex matched normal subjects.


Fenestration in the Fontan circulation as a strategy for chronic cardioprotection.

Saiki H, Kuwata S, Iwamoto Y, Ishido H, Taketazu M, Masutani S, Nishida T, Senzaki H.

Heart. 2019 Mar 2. pii: heartjnl-2018-314183. doi: 10.1136/heartjnl-2018-314183. [Epub ahead of print]

PMID: 30826770


Take Home Points: from Dr. Konstantin Averin (Edmonton)


·    Fenestration patency may confer hemodynamic advantages such as lower HR, lower CVP and a better preload reserve.

·    Providers should carefully consider the risks and benefits of elective fenestration occlusion prior to proceeding with closure.

·    Further study is indicated as to the long term risks and benefits of fenestration patency.

Congenital Cardiology Today - Assessment of Hemodynamics in Children with Complex Congenital Heart Disease Using a Micromanometer Pressure Wire

May 2019 Issue North American Edition

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Assessment of Hemodynamics in Children with Complex Congenital Heart Disease Using a Micromanometer Pressure Wire By Vishal R. Kaley, MBBS, MD; Chad B. Hopkins, BS; Harikrishnan K. N. Kurup, MBBS, MD; Bennett P. Samuel, MHA, BSN, RN; Yasser Al-Khatib, MD, FAAP, FACC, FSCAI; E. Oliver Aregullin, MD, FAAP; Joseph J. Vettukattil, MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP – p. 1
A Review of When I Wished Upon a Star; From Broken Homes to Mended Hearts By Virginia Dematatis – p. 8
Medical News, Products & Information – p. 12 Recruitment – p. 9, 11


Transition of Care for Congenital Heart Patients: Challenges, Opportunities, and
Pathways for Providers
By Wayne J. Franklin, MD – p. 109

ACHA Webinars for Patients and Families

Cardiovascular Health in ACHD Patients: Are You at Risk?

Wednesday, May 1, 2019, 7 p.m. – 8 p.m. EDT

**Please note the date has changed from the original date of April 24, 2019.**

Presented by George Lui, MD


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On behalf of the CHiP Network, thank you for reading!

Gary Webb, MD
CHiP Network