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Transthoracic ultrasonic tissue indices identify patients with severe left anterior descending artery stenosis. Correlation with fractional flow reserve. Pilot study.

Kardiol Pol. 2016;74(9):1010-5

Authors: Dobrowolski P, Kowalski M, Rybicka J, Lech A, Tyczyński P, Witkowski A, Hoffman P

BACKGROUND AND AIM: The aim of this study was to evaluate the potential clinical application of ultrasonic tissue indices, with a focus on systolic strain (SS) and systolic strain rate (SSR) parameters derived from transthoracic echocardiography, in the assessment of left anterior descending artery (LAD) stenosis.
METHODS: The data of 30 patients with significant LAD stenosis were analysed. All patients underwent transthoracic echocardiography to obtain systolic myocardial velocity (Sm), longitudinal SS, and SSR from basal, mid, and apical segments of anterior and inferior walls in two-chamber apical view. Severity of LAD obstruction was measured by means of fractional flow reserve (FFR) during coronary catheterisation.
RESULTS: Systolic velocities, strain, and strain rate measured in basal, middle, and apical segments of the anterior left ventricular (LV) wall were lower when compared to those obtained from the corresponding, i.e. unaffected, inferior LV wall. There was a significant correlation between FFR and the value of SS, SSR characterising the apical LV segment of the anterior wall (r = -0.583, p = 0.01; r = -0.598, p = 0.01, respectively). Moreover, we found significant correlation between FFR and Sm in the mid-segment of the LV anterior wall (r = 0.611, p = 0.009).
CONCLUSIONS: We conclude that SS and SSR obtained from the apical segment of the anterior LV wall may be related to the severity of LAD stenosis.

PMID: 27040014 [PubMed - indexed for MEDLINE]

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Conotruncal Heart Defect Repair in Sub-Saharan Africa: Remarkable Outcomes Despite Poor Access to Treatment.

World J Pediatr Congenit Heart Surg. 2016 Sep;7(5):592-9

Authors: Edwin F, Entsua-Mensah K, Sereboe LA, Tettey MM, Aniteye EA, Tamatey MM, Adzamli I, Akyaa-Yao N, Gyan KB, Ofosu-Appiah E, Kotei D

BACKGROUND: The outcome of children born with conotruncal heart defects may serve as an indication of the status of pediatric cardiac care in sub-Saharan Africa (SSA). This study was undertaken to determine the outcome of children born with conotruncal anomalies in SSA, regarding access to treatment and outcomes of surgical intervention.
METHODS: From our institution in Ghana, we retrospectively analyzed the outcomes of surgery, in the two-year period from June 2013 to May 2015. The birth prevalence of congenital heart defects (CHDs) in SSA countries was derived by extrapolation using an incidence of 8 per 1,000 live births for CHDs.
RESULTS: The birth prevalence of CHDs for the 48 countries in SSA using 2013 country data was 258,875; 10% of these are presumed to be conotruncal anomalies. Six countries (Nigeria, Democratic Republic of the Congo, Ethiopia, Tanzania, Uganda, and Kenya) accounted for 53.5% of the birth prevalence. In Ghana, 20 patients (tetralogy of Fallot [TOF], 17; pulmonary atresia, 3) underwent palliation and 50 (TOF, 36; double-outlet right ventricle, 14) underwent repair. Hospital mortality was 0% for palliation and 4% for repair. Only 6 (0.5%) of the expected 1,234 cases of conotruncal defects underwent palliation or repair within two years of birth.
CONCLUSION: Six countries in SSA account for more than 50% of the CHD burden. Access to treatment within two years of birth is probably <1%. The experience from Ghana demonstrates that remarkable surgical outcomes are achievable in low- to middle-income countries of SSA.

PMID: 27587494 [PubMed - indexed for MEDLINE]

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Quality of Life for Historic Cavopulmonary Shunt Survivors.

World J Pediatr Congenit Heart Surg. 2016 Sep;7(5):630-4

Authors: Steele MM, Zahr RA, Kirshbom PM, Kopf GS, Karimi M

BACKGROUND: Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort.
METHODS: Of the original 91 cavopulmonary shunt survivors, 14 (ages: 26-59; average length of postsurgical follow-up: 38.4 years) completed the Rand 36-Item Short-Form Health Survey, volume 2 (SF-36v2) quality-of-life survey at clinical office visits, over the phone or via regular mail. Diagnoses included tricuspid atresia (n = 9), double inlet left ventricle (n = 1), d-transposition of great arteries (n = 1), Ebstein's anomaly (n = 1), tetralogy of Fallot (n = 1), and pulmonary atresia with hypoplastic right ventricle (n = 1). Norm-based comparison of cavopulmonary shunt survivors to the general population was performed using a one-sample t test.
RESULTS: The 36-question health survey, SF-36v2, provided physical and mental health summary measures standardized to achieve a mean of 50. The 14 survivors' physical composite score was 47.18 (standard deviation [SD]: 8.24; P = .22) and mental composite score was 52.71 (SD: 5.64; P = .095).
CONCLUSION: The cavopulmonary shunt is now a widely used surgical palliation for single-ventricle patients and can be associated with physical and mental health outcomes similar to the general US population. Cavopulmonary shunt survivors' SF-36v2 survey results do not demonstrate a statistically significant difference from the general US population in physical or mental health measures. The results of this study contribute to the discussion of quality of life for patients with congenital heart disease by following up with the oldest known survivors.

PMID: 27587501 [PubMed - indexed for MEDLINE]

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Partial hydatidiform mole in a phenylketonuria patient treated with sapropterin dihydrochloride.

Gynecol Endocrinol. 2017 Jan;33(1):19-20

Authors: Yildiz Y, Dursun A, Tokatli A, Coskun T, Sivri S

Strict control of hyperphenylalaninemia is necessary in pregnant women with phenylketonuria (PKU) in order to prevent phenylalanine embryopathy in the fetus, characterized by intrauterine growth restriction, dysmorphic facies, congenital heart disease, microcephaly and intellectual disability, collectively known as maternal PKU syndrome. Sapropterin dihydrochloride (SD), an alternative or adjunct to dietary therapy in patients with tetrahydrobiopterin (BH4)-responsive PKU, has recently been used in several cases to treat PKU during pregnancy with satisfactory results. Here, we report two pregnancies treated with SD and unrestricted diet in a patient with BH4-responsive mild PKU. The first pregnancy resulted in a partial hydatidiform mole and was terminated, whereas a healthy infant was born from the second pregnancy. Phenylalanine control was optimal in both pregnancies. To the best of our knowledge, this is the first report on the development of partial hydatidiform mole associated with SD treatment and the second report on molar pregnancy in PKU. While the relation between SD and molar pregnancy is unknown, further studies may be needed to investigate the possible effects of SD on fertilization.

PMID: 27898272 [PubMed - indexed for MEDLINE]

Transition in Patients with Congenital Heart Disease in Germany: Results of a Nationwide Patient Survey.

Front Pediatr. 2017;5:115

Authors: Helm PC, Kaemmerer H, Breithardt G, Sticker EJ, Keuchen R, Neidenbach R, Diller GP, Tutarel O, Bauer UMM

BACKGROUND: A growing number of adults with congenital heart disease (ACHD) pose a particular challenge for health care systems across the world. Upon turning into 18 years, under the German national health care system, ACHD patients are required to switch from a pediatric to an adult cardiologist or an ACHD-certified provider. To date, reliable data investigating the treatment situation of ACHD patients in Germany are not available.
MATERIALS AND METHODS: An online survey was conducted in collaboration with patient organizations to address the life situation and the conditions of health care provision for ACHD patients in Germany. ACHD patients were recruited from the database of the National Register for Congenital Heart Defects (NRCHD) and informed about the survey via email, websites, and social networks. A total of 1,828 ACHD patients (1,051 females) participated in this study. The mean age was 31.7 ± 11.7 years. Participants were surveyed about treating physicians and the institution mainly involved in the treatment of their CHD. In addition, participants were asked questions to assess the level of trust toward their treating physician and their familiarity with the term "ACHD-certified provider."
RESULTS: Among the surveyed patients, 25.4% stated that they attended a specific ACHD clinic at a heart center regularly, 32.7% were treated in a private practice setting by a pediatric cardiologist, 32.4% in a private practice (adult) cardiology setting, and 9.5% were treated by an "other physician." Only 24.4% of the male and 29.7% of the female ACHD patients were familiar with the term "ACHD-certified provider."
CONCLUSION: The transfer from pediatric cardiology to ACHD care requires further attention as many adult patients have not transferred to certified ACHD providers. The question of whether ACHD patients in Germany are offered consistent and adequate care should also be investigated in more detail. The answers regarding the ACHD certification are particularly disappointing and indicative of a large information gap and inadequate education in clinical practice.

PMID: 28580351 [PubMed - in process]

Improving the quality of transition and transfer of care in young adults with congenital heart disease.

Congenit Heart Dis. 2017 May;12(3):242-250

Authors: Everitt IK, Gerardin JF, Rodriguez FH, Book WM

The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease-specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients.

PMID: 28580608 [PubMed - in process]

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[Efficacy and safety of balloon valvuloplasty as a treatment of choice for pulmonary stenosis in children and adolescents].

Srp Arh Celok Lek. 2014 Sep-Oct;142(9-10):542-6


INTRODUCTION: Pulmonary artery stenosis (PS) is a congenital heart defect which occurs in 10% of all congenital heart defects. Pulmonary balloon valvuloplasty (BVP) has been the treatment of choice of PS over the last 30 years.
OBJECTIVE: The purpose of this study was to evaluate the effi- cacy of this method based on middle-term hospital follow-up, and safety of BVP based on our experience.
METHODS: The study included 88 patients diagnosed with PS.The patients were divided into three groups based on the severity of the disease. Also, they were divided into two age groups in order to analyze the frequency of complications. Hemodynamic measurements and echocardiography results were recorded before, 24-36 hours after BVP and at the end of follow-up.
RESULTS: The studied group involved patients of average age 3.75 ± 4.3 years (20 days to 17 years). Immediately after BVP a significant decrease of pressure gradient across the pulmonary valve (PV) was recorded in all patients; this result was similar in all 3 groups of patients regardless of the severity of stenosis (p < 0.001). Complications of BVP occurred most commonly in children up to 12 months of age (ventricular tachycardia 4.5% and supraventricular tachycardia 6.8%). Pulmonary valve in- sufficiency after dilatation occurred in 6.6% of cases, and was most common in children aged up to 12 months. In 87 (98.9%) patients BVP was a definitive solution, and a significant residual stenosis was not recorded during follow-up.
CONCLUSION: BVP is a safe and effective procedure in the treatment of isolated PS in children, regardless of the severity of stenosis but also regardless of patients' age.

PMID: 25518531 [PubMed - indexed for MEDLINE]

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Quality of Life of Adults With Congenital Heart Disease in 15 Countries: Evaluating Country-Specific Characteristics.

J Am Coll Cardiol. 2016 May 17;67(19):2237-45

Authors: Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Chidambarathanu S, Alday L, Eriksen K, Dellborg M, Berghammer M, Mattsson E, Mackie AS, Menahem S, Caruana M, Veldtman G, Soufi A, Romfh AW, White K, Callus E, Kutty S, Fieuws S, Moons P, APPROACH-IS consortium and ISACHD

BACKGROUND: Measuring quality of life (QOL) is fundamental to understanding the impact of disease and treatment on patients' lives.
OBJECTIVES: This study aimed to explore QOL in an international sample of adults with congenital heart disease (CHD), the association between patient characteristics and QOL, and international variation in QOL and its relationship to country-specific characteristics.
METHODS: We enrolled 4,028 adults with CHD from 15 countries. QOL was assessed using a linear analog scale (LAS) (0 to 100) and the Satisfaction with Life Scale (SWLS) (5 to 35). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, and patient-reported New York Heart Association (NYHA) functional class. Country-specific characteristics included general happiness and 6 cultural dimensions. Linear mixed models were applied.
RESULTS: Median QOL was 80 on the LAS and 27 on the SWLS. Older age, lack of employment, no marriage history, and worse NYHA functional class were associated with lower QOL (p < 0.001). Patients from Australia had the highest QOL (LAS: 82) and patients from Japan the lowest (LAS: 72). Happiness scores and cultural dimensions were not associated with variation in QOL after adjustment for patient characteristics and explained only an additional 0.1% of the variance above and beyond patient characteristics (p = 0.56).
CONCLUSIONS: This large-scale, international study found that overall QOL in adults with CHD was generally good. Variation in QOL was related to patient characteristics but not country-specific characteristics. Hence, patients at risk for poorer QOL can be identified using uniform criteria. General principles for designing interventions to improve QOL can be developed.

PMID: 27173035 [PubMed - indexed for MEDLINE]

Related Articles

Aortic Valve Replacement and the Ross Operation in Children and Young Adults.

J Am Coll Cardiol. 2016 Jun 21;67(24):2858-70

Authors: Sharabiani MT, Dorobantu DM, Mahani AS, Turner M, Peter Tometzki AJ, Angelini GD, Parry AJ, Caputo M, Stoica SC

BACKGROUND: There are several options available for aortic valve replacement (AVR), with few comparative reports in the literature. The optimal choice for AVR in each age group is not clear.
OBJECTIVES: The study sought to report and compare outcomes after AVR in the young using data from a national database.
METHODS: AVR procedures were compared after advanced matching, both in pairs and in a 3-way manner, using a Bayesian dynamic survival model.
RESULTS: A total of 1,501 patients who underwent AVR in the United Kingdom between 2000 and 2012 were included. Of these, 47.8% had a Ross procedure, 37.8% a mechanical AVR, 10.9% a bioprosthesis AVR, and 3.5% a homograft AVR, with Ross patients being significantly younger when compared to the other groups. Overall survival at 12 years was 94.6%. In children, the Ross procedure had a 12.7% higher event-free probability (death or any reintervention) at 10 years when compared to mechanical AVR (p = 0.05). We also compared all procedures except the homograft in a matched population of young adults, where the bioprosthesis had the lowest event-free probability of 78.8%, followed by comparable results in mechanical AVR and Ross, with 86.3% and 89.6%, respectively. Younger age was associated with mortality and pulmonary reintervention in the Ross group and with aortic reintervention in the mechanical AVR. Of all 3 options, only the patients undergoing the Ross procedure approached the survival of the general population.
CONCLUSIONS: AVR in the young achieves good results, with the Ross being overall better suited for this age group, especially in children. Although freedom from aortic valve reintervention is superior after the Ross procedure, the need for homograft reinterventions is an issue to take into account. All methods have advantages and limitations, with reinterventions being an issue in the long term for all, more crucially in smaller children.

PMID: 27311525 [PubMed - indexed for MEDLINE]

Related Articles

Risk factors for pulmonary arterial hypertension in children and young adults.

Pediatr Pulmonol. 2017 May;52(5):636-641

Authors: Naumburg E, Söderström L, Huber D, Axelsson I

OBJECTIVES: Pulmonary hypertension (PH) has been linked to preterm birth explained by congenital heart defects and pulmonary diseases.
WORKING HYPOTHESIS: Other factors may influence the risk of PH among adolescences and children born premature.
STUDY DESIGN: This national registry-based study assess risk of PH following premature birth adjusted for known risk factors.
PATIENT-SUBJECT SELECTION AND METHODOLOGY: All cases born 1993-2010, identified by diagnostic codes applicable to PH and retrieved from the Swedish Registry of Congenital Heart Disease (N = 67). Six controls were randomly selected and matched to each case by year of birth and hospital by the Swedish Medical Birth Register (N = 402). Maternal and infant data related to preterm birth, pulmonary diseases, and congenital defects were retrieved. The association between preterm birth and pulmonary hypertension was calculated by conditional logistic regression taking into account potential confounding factors.
RESULTS: One third of the cases and seven percent of the controls were born preterm in our study. Preterm birth was associated with PH, OR = 8.46 (95%CI 2.97-24.10) (P < 0.0001) even after adjusting for confounding factors. Other factors, such as acute pulmonary diseases, congenital heart defects, congenital diaphragm herniation, and chromosomal disorders were also associated with PH in the multivariate analysis.
CONCLUSIONS: Children and young adults born preterm are known to have an increased risk of PH, previously explained by congenital heart defects and pulmonary diseases. By adjusting for such factors, our study indicates that new factors may play a role in the risk of developing PH among children born preterm. Pediatr Pulmonol. 2017;52:636-641. © 2016 Wiley Periodicals, Inc.

PMID: 27801982 [PubMed - indexed for MEDLINE]