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Atrial arrhythmias in adults with congenital heart disease. Listening to your heart sound can save your life.

Int J Cardiol. 2017 12 01;248:159-160

Authors: Limongelli G, Sarubbi B

PMID: 28942871 [PubMed - indexed for MEDLINE]

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Characteristics and Prognosis of Pacemaker-Identified New-Onset Atrial Fibrillation in Japanese People.

Circ J. 2017 May 25;81(6):794-798

Authors: Ogino Y, Ishikawa T, Ishigami T, Matsumoto K, Hosoda J, Iguchi K, Matsushita H, Taguchi Y, Horiguchi Y, Kimura K

BACKGROUND: The characteristics and prognosis of implanted pacemaker-identified new-onset atrial fibrillation (AF) in Japanese people has not been well evaluated.Methods and Results:A total of 395 consecutive patients with newly implanted pacemakers were retrospectively analyzed between January 2010 and December 2015 at Yokohama City University Hospital. Patients with a prior history of AF, VVI mode pacemaker, congenital heart disease, severe valvular heart disease, and cardiovascular surgery before pacemaker implantation were excluded. Among the remaining patients, 44 (21.3%) developed new AF during follow-up (mean follow-up, 1,115±651 days; range, 9-2,176 days). Patients with new-onset AF had a significantly higher CHADS2score (2.09±1.27 vs. 1.31±1.08, P<0.001) and CHA2DS2-VASc score (3.00±1.39 vs. 2.26±1.19, P<0.001) compared with those without new-onset AF. On Cox regression analysis only age at implantation was significantly correlated with new-onset AF. Interestingly, the incidence of hospitalization due to heart failure was significantly higher in the new-onset AF than in the without new-onset AF group.
CONCLUSIONS: A total of 21.3% of pacemaker-implanted patients with high CHADS2and CHA2DS2-VASc scores developed new-onset AF during a mean follow-up of 3.1 years; and pacemaker-identified AF was associated with an increased risk of worsening heart failure.

PMID: 28250283 [PubMed - indexed for MEDLINE]

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Transcatheter valve replacement in congenital heart disease: the present and the future.

Heart. 2018 Feb 28;:

Authors: Ghobrial J, Aboulhosn J

PMID: 29490935 [PubMed - as supplied by publisher]

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Overview of adult congenital heart transplants.

Ann Cardiothorac Surg. 2018 Jan;7(1):143-151

Authors: Bryant R, Morales D

Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, the indications and potential contraindications for transplant, surgical considerations, and post-transplant outcomes. The findings reveal that 3.3% of adult heart transplants occur in ACHD patients. The potential mechanisms for the development of late ventricular dysfunction include a morphologic systemic right ventricle, altered coronary perfusion, and ventricular noncompaction. The indications for transplant in ACHD patients include systemic ventricular failure refractory medical therapy, Fontan patients failing from chronic passive pulmonary circulation, and progressive cyanosis leading to functional decline. Transplantation in ACHD patients can be quite complex and may require extensive reconstruction of the branch pulmonary arteries, systemic veins, or the aorta. Vasoplegia, bleeding, and graft right ventricular dysfunction can complicate the immediate post-transplant period. The post-transplant operative mortality ranges between 14% and 39%. The majority of early mortality occurs in ACHD patients with univentricular congenital heart disease. However, there has been improvement in operative survival in more contemporary studies. In conclusion, the experience with cardiac transplantation for ACHD patients with end-stage heart failure is growing, and high-risk patient subsets have been defined. Significant strides have been made in developing evidence-based guidelines of indications for transplant, and the intraoperative management of complex reconstruction has evolved. With proper patient selection, more aggressive use of mechanical circulatory support, and earlier referral of patients with failing Fontan physiology, outcomes should continue to improve.

PMID: 29492392 [PubMed]

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Transplantation in the single ventricle population.

Ann Cardiothorac Surg. 2018 Jan;7(1):152-159

Authors: Kenny LA, DeRita F, Nassar M, Dark J, Coats L, Hasan A

The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair. Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multi-disciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.

PMID: 29492393 [PubMed]

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Inter- and intra-ventricular dyssynchrony in the systemic right ventricle is a surrogate marker of major cardiac events in mildly symptomatic patients.

Heart Vessels. 2018 Feb 28;:

Authors: Shiina Y, Inai K, Takahashi T, Taniguchi K, Watanabe E, Fukushima K, Niwa K, Nagao M

The aim of the study was to evaluate systemic right ventricular (RV) dyssynchrony in patients with congenitally corrected transposition of the great arteries (CCTGA) and transposition of the great arteries (TGA) with New York Heart Association functional class (NYHA FC) < III. We used cardiac magnetic resonance (CMR) to evaluate the dyssynchrony and assessed whether RV dyssynchrony can be predictive of major cardiac events in their early stages in these patients. We enrolled 71 consecutive, NYHA FC < III patients with systemic RV who underwent CMR between April 1995 and December 2016. We measured intra- and inter-ventricular dyssynchrony using a feature-tracking method of cine magnetic resonance imaging. The predictors of major cardiac events were analyzed using the Cox hazard analysis. The data from 36 patients with CCTGA and 35 patients with TGA after an atrial switch were analyzed. Seven (19.4%) patients with CCTGA and 6 (17.1%) patients with TGA showed a QRS duration of ≥ 130 ms. There were significant intra- and inter-dyssynchrony in the systemic RV groups, compared to healthy controls. The average follow-up period was 5.1 ± 3.9 years. From among patients with CCTGA, 9 (25.0%) had major cardiac events. The parameters including NYHA FC, indexed RV volume, longitudinal early diastolic strain rate, and intra- and inter-ventricular dyssynchrony were predictive of major cardiac events. From among patients with TGA, 12 (34.3%) had major cardiac events. Age, NYHA FC, QRS duration, RV volume, RV mass index, LV volume, global longitudinal/circumferential strain and intraventricular dyssynchrony, were all predictive of major cardiac events. Systemic RV in NYHA FC < III patients with CCTGA and TGA, have obvious intra- and inter-dyssynchrony, suggesting ineffective wall motion and potential RV dysfunction. Intraventricular dyssynchrony can be an adjunct predictor of major cardiac events in mildly symptomatic patients with both CCTGA and TGA.

PMID: 29492650 [PubMed - as supplied by publisher]

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Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

J Heart Lung Transplant. 2017 Apr;36(4):386-398

Authors: Hascoët S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, Laux D, Sitbon O, Lambert V, Capderou A

BACKGROUND: The long-term effectiveness of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort.
METHODS: Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) >8 WU·m2, and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification. Oxygen consumption was measured and the Fick principle applied to calculate indexed pulmonary output (Qpi) and PVRi.
RESULTS: After PAH-SDT initiation or intensification, median (interquartile range) PVRi decrease was 5.1 WU·m2 (-1.4, -12.6) (p < 0.0001). Median Qpi and 6-minute walk test increases were +0.4 liter/min/m2 (0.0, +0.9) (p < 0.0001) and +49 m (+15, +93) (p = 0.0003), respectively. Hemodynamic response combining increased Qpi with decreases in transpulmonary gradient and PVRi occurred in 68.0% of patients. After a median of 4.9 years, PVRi and Qpi changes were no longer significant. Over a median of 7.2 years, 23 (33.3%) patients met a composite criterion (death, n = 8; heart-lung transplantation or listing for transplantation, n = 15). The 15-year cumulative event rate was 49.2%. By multivariate analysis, independent predictors of events were superior vena cava oxygen saturation and hemodynamic response (p = 0.048 and p < 0.0001).
CONCLUSIONS: In Eisenmenger syndrome, PAH-SDT induces early hemodynamic improvements, which decline over time. Hemodynamic changes under PAH-SDT vary across patients. Hemodynamic parameters at baseline and under PAH-SDT are associated with events. PAH-SDT may need to be individualized based on hemodynamic changes.

PMID: 27866929 [PubMed - indexed for MEDLINE]

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Single-center outcomes of combined heart and liver transplantation in the failing Fontan.

Clin Transplant. 2017 Mar;31(3):

Authors: D'Souza BA, Fuller S, Gleason LP, Hornsby N, Wald J, Krok K, Shaked A, Goldberg LR, Pochettino A, Olthoff KM, Kim YY

Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed. Of 17 referrals for cardiac transplantation, seven Fontan patients underwent CHLT. All patients who underwent CHLT had either advanced fibrosis or cirrhosis on liver biopsy. There were no perioperative deaths. The most common postoperative morbidity was acute kidney injury. Short-term complications include one episode of acute liver rejection but no cardiac rejection greater than 1R. CHLT is an acceptable therapeutic option for patients with failing Fontan physiology who exhibit concomitant advanced liver fibrosis. However, optimal patient selection is currently undefined, and long-term outcomes are not known.

PMID: 27988989 [PubMed - indexed for MEDLINE]

Clinical Outcomes in Adolescents and Adults After the Fontan Procedure.

J Am Coll Cardiol. 2018 Mar 06;71(9):1009-1017

Authors: Dennis M, Zannino D, du Plessis K, Bullock A, Disney PJS, Radford DJ, Hornung T, Grigg L, Cordina R, d'Udekem Y, Celermajer DS

BACKGROUND: Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers.
OBJECTIVES: This study sought to describe the survival and complications of adult patients who have had a Fontan procedure.
METHODS: The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR.
RESULTS: Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age.
CONCLUSIONS: This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.

PMID: 29495980 [PubMed - in process]

The Adolescent and Adult With a Fontan Circulation: "Unnatural" Selection and Survival of the Fittest.

J Am Coll Cardiol. 2018 Mar 06;71(9):1018-1020

Authors: Rychik J, Kim Y

PMID: 29495981 [PubMed - in process]