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Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD).

Int J Cardiol. 2018 Jul 11;:

Authors: Li W, West C, McGhie J, van den Bosch AE, Babu-Narayan SV, Meijboom F, Mongeon FP, Khairy P, Kimball TR, Beauchesne LM, Ammash NM, Veldtman GR, Oechslin E, Gatzoulis MA, Webb G

The population of adults with congenital heart disease (ACHD) is increasing constantly due to medical, surgical and interventional successes and the input from advanced cardiovascular imaging. ACHD patients are at continuing risk of residua and sequelae related to their CHD contributing to significant morbidity and mortality. Consequently, lifelong expert surveillance is recommended for most patients. Healthcare providers are still working out how best to achieve this objective, how to train enough experts to provide high quality care, and how to organize the delivery of care. Echocardiography is crucial to clinical surveillance providing a comprehensive assessment of cardiac morphology, physiology, pathophysiology, and function. Thus it contributes significantly to the overall clinical management of ACHD patients. The International Society for Adult Congenital Heart Disease (ISACHD; is the leading organization of professionals worldwide dedicated to the pursuit of excellence in the care of ACHD patients. Recognizing the critical role of imaging in the diagnosis and management of ACHD, ISACHD established a task force to provide guidance on echocardiographic studies and reporting. The rationale is that standardization of echocardiographic imaging and reporting carries the potential to improve the overall quality of these exams around the world and facilitate collaborative multicenter research. The standardized ACHD protocols provided by the ISACHD task force (found in the appendices) include specific recommendations for data acquisition and reporting for each of the major adult congenital heart lesions. These protocols give a comprehensive and structured approach in the evaluation of ACHD patients and help to ensure excellent patient care.

PMID: 30017529 [PubMed - as supplied by publisher]

What is the role of apical ventriculotomy in children and young adults with hypertrophic cardiomyopathy?

Congenit Heart Dis. 2018 Jul 17;:

Authors: Thompson AJ, Dearani JA, Johnson JN, Schaff HV, Towe EC, Palfreeman J, Wackel PL, Cetta F

BACKGROUND: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.
METHODS: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 ± 4.0 years (range, 4-20).
RESULTS: Overall, 23 patients (12 males) underwent transapical myectomy. A concomitant transaortic approach was performed in 16/19 (84%) with obstruction. The intraventricular gradient decreased from 71 mm Hg (IQR 44-92 mm Hg) preoperatively to 18 mm Hg (IQR 8-34 mm Hg, P < .0001) after myectomy. In patients with a small LV cavity, the mean left ventricular end diastolic dimension (LVEDD) increased from 40 ± 3 mm to 46 ± 3 mm (P = .05) after myectomy. There were no early deaths. Postoperative morbidity included complete heart block in 3 patients, 2 of which required pacemakers. Median follow up was 3.5 years (IQR 1.6-5.6). Symptoms improved in 95% of patients; the number of patients in NYHA class 3 or 4 heart failure decreased from 10/23 (43%) preoperatively to 3/23 (13%) postoperatively (P < .0001). Overall survival at 5 years postsurgery was 100%. Transplant-free survival was 91% and 87% at 1 and 5 years, respectively.
CONCLUSION: In children with HCM, transapical myectomy is an effective adjunct to a transaortic approach to abolish midventricular obstruction and it effectively increases LV stroke volume in patients with small LV cavities and nonobstructive HCM. It may be beneficial for these patients with significant symptoms and who have failed medical therapy as a treatment alternative to cardiac transplantation.

PMID: 30019505 [PubMed - as supplied by publisher]

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Patient-reported outcomes in adults with congenital heart disease: Inter-country variation, standard of living and healthcare system factors.

Int J Cardiol. 2018 Jan 15;251:34-41

Authors: Moons P, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Yang HL, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Mackie AS, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, White K, Callus E, Kutty S, Van Bulck L, Apers S, APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD)

AIMS: Geographical differences in patient-reported outcomes (PROs) of adults with congenital heart disease (ConHD) have been observed, but are poorly understood. We aimed to: (1) investigate inter-country variation in PROs in adults with ConHD; (2) identify patient-related predictors of PROs; and (3) explore standard of living and healthcare system characteristics as predictors of PROs.
METHODS AND RESULTS: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS) was a cross-sectional, observational study, in which 4028 patients from 15 countries in 5 continents were enrolled. Self-report questionnaires were administered: patient-reported health (12-item Short Form Health Survey; EuroQOL-5D Visual Analog Scale); psychological functioning (Hospital Anxiety and Depression Scale); health behaviors (Health Behavior Scale-Congenital Heart Disease) and quality of life (Linear Analog Scale for quality of life; Satisfaction With Life Scale). A composite PRO score was calculated. Standard of living was expressed as Gross Domestic Product per capita and Human Development Index. Healthcare systems were operationalized as the total health expenditure per capita and the overall health system performance. Substantial inter-country variation in PROs was observed, with Switzerland having the highest composite PRO score (81.0) and India the lowest (71.3). Functional class, age, and unemployment status were patient-related factors that independently and consistently predicted PROs. Standard of living and healthcare system characteristics predicted PROs above and beyond patient characteristics.
CONCLUSIONS: This international collaboration allowed us to determine that PROs in ConHD vary as a function of patient-related factors as well as the countries in which patients live.

PMID: 29107358 [PubMed - indexed for MEDLINE]

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Resynchronization Therapy for Patients with Congenital Heart Disease: Are We Ready for Prime Time?

Curr Cardiol Rep. 2018 Jul 18;20(9):75

Authors: Anjewierden S, Aziz PF

PURPOSE OF REVIEW: This review aims to summarize and provide an update of evidence for cardiac resynchronization therapy (CRT) in pediatric and adult congenital heart disease (CHD).
RECENT FINDINGS: CRT is a valuable tool in the treatment of heart failure in the setting of CHD. Current evidence points toward the ability of this therapy to increase hemodynamic parameters and reduce heart failure-related symptoms. Within the CHD population, patients with systemic left ventricles appear to benefit the most from CRT. Utilization of CRT in CHD has been technically difficult due to the complexity and variety of anatomic substrates. However, lateral thoracotomies and other advances have made implantation more feasible. The most up-to-date evidence continues to support the use of CRT in the heterogeneous population of CHD with careful and individualized patient selection. Further studies are needed to evaluate the utility of CRT for specific anatomic substrates within this population.

PMID: 30022366 [PubMed - in process]

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Coping with the Stress in the Cardiac Intensive Care Unit: Can Mindfulness Be the Answer?

J Pediatr Nurs. 2017 Nov - Dec;37:117-126

Authors: Golfenshtein N, Deatrick JA, Lisanti AJ, Medoff-Cooper B

BACKGROUND: Mothers of infants with complex congenital heart disease are exposed to increased stress which has been associated with numerous adverse health outcomes. The coping mechanisms these mothers use critically effect the familial illness adaptation and most likely infant outcomes. Currently no data-based strategies have been developed for mothers to facilitate their coping, and proactively promote their adaptation in the critical care settings. A potential strategy is mindfulness which is currently used in other clinical settings with stress-reduction effects.
PURPOSE: (1) To investigate coping mechanisms of mothers whose infant with complex CHD is admitted in the CICU, and (2) to explore the acceptability and feasibility of mindfulness as a potential stress-reduction intervention for these mothers.
DESIGN AND METHODS: A descriptive qualitative study obtained perspectives from 14 mothers during three focus groups. A qualitative conventional content analysis was performed using ATLAS.ti.
RESULTS: In congruence with the Stress and Coping framework, themes identified mostly emotion-regulatory coping mechanisms including both active and passive strategies such as positive thinking, denial, distraction, relying on support systems, and focusing on baby. Mindfulness was an acceptable and feasible approach for most participants, however, practice unfamiliarity, time and space concerns, and personal preferences were identified as potential barriers for future dissemination.
CONCLUSIONS: Mindfulness can potentially promote illness adaptation by utilization of active coping mechanisms. Early interventions can provide immediate, and potentially long-term stress relief. Intervention settings, format, and time-frame should be flexibly tailored to the trajectory of parental distress and familial adjustment.

PMID: 28807423 [PubMed - indexed for MEDLINE]

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Risk of Infective Endocarditis in Patients with End Stage Renal Disease.

Clin J Am Soc Nephrol. 2017 Nov 07;12(11):1814-1822

Authors: Chaudry MS, Carlson N, Gislason GH, Kamper AL, Rix M, Fowler VG, Torp-Pedersen C, Bruun NE

BACKGROUND AND OBJECTIVES: Endocarditis is a serious complication in patients treated with RRT. The study aimed to examine incidence and risk factors of endocarditis in patients with ESRD.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The Danish National Registry on Regular Dialysis and Transplantation contains data on all Danish patients receiving renal replacement (hemodialysis, peritoneal dialysis, or kidney transplantation) for ESRD. Incidence of endocarditis was estimated for each RRT modality. Independent risk factors of endocarditis were identified in multivariable Cox regression models.
RESULTS: From January 1st, 1996 to December 31st, 2012, 10,612 patients (mean age 63 years, 36% female) initiated RRT (7233 hemodialysis, 3056 peritoneal dialysis, 323 pre-emptive kidney transplantation). Endocarditis developed in 267 (2.5%); of these 31 (12%) underwent valve surgery. The overall incidence of endocarditis was 627 per 100,000 person-years in patients receiving RRT. Incidence was higher in patients receiving hemodialysis compared with those receiving peritoneal dialysis or kidney transplantation (1092 per 100,000 person-years, 212 per 100,000 person-years, and 85 per 100,000 person-years, respectively). Adjusted hazard ratios for endocarditis in patients receiving hemodialysis were 5.46 (95% confidence interval [95% CI], 3.28 to 9.10) and 0.41 (95% CI, 0.18 to 0.91) for kidney-transplanted recipients, respectively, as compared with patients in peritoneal dialysis. The incidence of endocarditis in hemodialysis recipients with central venous catheters was more than two-fold higher as compared with those with arteriovenous fistulas. Overall mortality, subsequent to endocarditis, was 22% in-hospital and 51% at 1 year. The first 6 months in RRT, aortic valve disease, and previous endocarditis were identified as significant risk factors of endocarditis.
CONCLUSIONS: Patients receiving RRT have a high incidence of endocarditis, in particular during hemodialysis treatment using central venous catheters. The first 6 months in RRT, aortic valve disease, and previous endocarditis are significant risk factors for developing endocarditis.

PMID: 28974524 [PubMed - indexed for MEDLINE]

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Verification of Heart Disease: Implications for a New Heart Transplantation Allocation System.

JACC Heart Fail. 2017 Dec;5(12):904-913

Authors: Raeisi-Giglou P, Rodriguez ER, Blackstone EH, Tan CD, Hsich EM

OBJECTIVES: This study sought to determine the accuracy of the pre-transplantation clinical diagnosis of heart disease in the United Network for Organ Sharing (UNOS) database.
BACKGROUND: Because survival on the heart transplantation waitlist depends on underlying heart disease, a new allocation system will include the type of heart disease. Accuracy of the pre-transplantation clinical diagnosis and the effect of misclassification are unknown.
METHODS: We included all adults who received transplants at our center between January 2009 to December 2015. We compared the pre-transplantation clinical diagnosis at listing with pathology of the explanted heart and determined the potential effect of misclassification with the proposed allocation system.
RESULTS: A total of 334 patients had the following clinical cardiac diagnoses at listing: 148 had dilated cardiomyopathy, 19 had restrictive cardiomyopathy, 103 had ischemic cardiomyopathy, 24 had hypertrophic cardiomyopathy, 11 had valvular disease, 16 had congenital heart disease (CHD), and 13 patients had a diagnosis of "other." Pathology of the explanted hearts revealed 82% concordance and 18% discordance (10% coding errors and 8% incorrect diagnosis). The most common incorrect diagnoses were sarcoidosis (66%), arrhythmogenic right ventricular dysplasia (60%), and other causes of predominately right-sided heart failure (33%). Among the misclassified diagnoses, 40% were listed as UNOS status 2, 8% remained at status 2 at transplantation, and only sarcoidosis and CHD were potentially at a disadvantage with the new allocation.
CONCLUSIONS: There is high concordance between clinical and pathologic diagnosis, except for sarcoidosis and genetic diseases. Few misclassifications result in disadvantages to patients based on the new allocation system, but rare diseases like sarcoidosis remain problematic. To improve the UNOS database and enhance outcome research, pathology of the explanted hearts should be required post-transplantation.

PMID: 29191297 [PubMed - indexed for MEDLINE]

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Low Nasal NO in Congenital Heart Disease With Systemic Right Ventricle and Postcardiac Transplantation.

J Am Heart Assoc. 2017 Dec 06;6(12):

Authors: Adams PS, Zahid M, Khalifa O, Feingold B, Lo CW

BACKGROUND: NO bioavailability has not been systematically examined in congenital heart disease (CHD). To assess NO in patients with CHD, we measured nasal NO (nNO) generated by the nasal epithelia, given blood NO is difficult to measure (half-life, <2 ms). Given NO's role in hemodynamic regulation and the association of NO bioavailability with heart failure risk, we hypothesized NO levels may differ with varying severity of CHD physiologic characteristics.
METHODS AND RESULTS: Six-hundred eighteen subjects, 483 with CHD and 135 controls, had nNO measured noninvasively via the nares using American Thoracic Society/European Respiratory Society guidelines. Subjects were dichotomized as having low or normal nNO based on age-specific cutoff values. Prevalence of low nNO was examined by various CHD physiologic feature types. Low nNO was more prevalent with CHD than controls (odds ratio, 2.28; P=0.001). A logistic regression model showed overall significance (P=0.035) for single ventricle, systemic right ventricle, ventricular dysfunction, oxygen desaturation, and heterotaxy predicting low nNO, with systemic right ventricle independently having twice the odds of low nNO (odds ratio, 2.04; P=0.014). Patients with low nNO had a higher risk of experiencing heart transplant or death (hazard ratio, 2.75; P=0.048), and heart transplant recipients (N=16) exhibited 5 times the odds of low nNO (69% versus 30%; odds ratio, 5.1; P=0.001).
CONCLUSIONS: Patients with CHD have increased prevalence of low nNO, with highest odds seen with systemic right ventricle and heart transplant. Further studies are needed to investigate heart failure risks in patients with CHD with left versus right systemic ventricle physiologic characteristics and utility of low nNO for predicting heart failure risk.

PMID: 29212650 [PubMed - indexed for MEDLINE]

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Bioresorbable Scaffolds in Spontaneous Coronary Artery Dissection: Long-Term Follow-Up in 4 Patients.

Tex Heart Inst J. 2017 12;44(6):405-410

Authors: Camacho Freire SJ, Gómez Menchero AE, Roa Garrido J, León Jiménez J, Cardenal Piris R, Díaz Fernández JF

Spontaneous coronary artery dissection is a rare condition, and diagnosis and treatment are challenging among patients who present with acute coronary syndrome. Typically, the condition affects young females who have no underlying atherosclerotic disease. To date, few cases of bioresorbable scaffold implantation for the treatment of spontaneous coronary artery dissection have been reported. Therefore, we describe the cases of 4 patients whom we treated with scaffolds. We evaluated the long-term results by using intravascular ultrasound and optical coherence tomographic scanning.

PMID: 29276441 [PubMed - indexed for MEDLINE]

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Ruptured Sinus of Valsalva Aneurysm Associated with Situs Ambiguus, Isolated Levocardia, and Polysplenia.

Tex Heart Inst J. 2017 12;44(6):416-419

Authors: Golestaneh S, Kashef MA, Hiser WL, Lotfi AS, Egan TG

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.

PMID: 29276443 [PubMed - indexed for MEDLINE]