Journal Watch

Computed Tomography Appearance of Uncorrected Congenital Heart Disease in Adults.

J Thorac Imaging. 2019 Feb 07;:

Authors: Maldjian PD, Yen LH

The incidence of congenital heart disease (CHD) is about 4 to 10 per 1000 live births. While severe forms of CHD are usually diagnosed in infancy or childhood, milder and more survivable CHD may first manifest in the adult or may be discovered incidentally on imaging studies performed for other reasons. It is important to identify CHD in adults to implement appropriate treatment and prevent complications such as progression of pulmonary hypertension and development of heart failure. The goals of this article are to illustrate the appearance on computed tomography of various forms of uncorrected CHD in adults and describe the key features that characterize specific lesions.

PMID: 30741756 [PubMed - as supplied by publisher]

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Outcomes and trends of peripartum maternal admission to the intensive care unit.

Wien Klin Wochenschr. 2017 Sep;129(17-18):605-611

Authors: Farr A, Lenz-Gebhart A, Einig S, Ortner C, Holzer I, Elhenicky M, Husslein PW, Lehner R

BACKGROUND: The number of pregnant women with severe comorbidities is increasing. The aim of the present study was to analyze outcomes and determine trends in women who required peripartum admission to the intensive care unit (ICU).
METHODS: In this retrospective study, we identified all women who were admitted to the ICU between the second trimester of pregnancy and 6 weeks postpartum. Women with ICU admission between 2011 and 2014 were assigned to the study group, whereas those admitted between 1996 and 2003 were assigned to the historical group. Comorbidities, characteristics, outcomes, and treatment efforts were assessed. Descriptive analysis, Fisher's exact test, unpaired Student's t-test and one-way ANOVA were performed.
RESULTS: We identified 238 cases, including 135 (56.7%) in the study group and 103 (43.3%) in the historical group. In 83 (34.9%) women, deterioration of the pre-existing medical condition was causal for ICU admission. Overall, preterm delivery and mean gestational age were 81.5% and 31.6 ± 6.2 weeks, respectively. In comparison to the historical group, women of the study group were older (p = 0.005), more frequently presented with multiple comorbidities (p = 0.003), pre-existing conditions (p < 0.001), and congenital heart disease (p = 0.012). Moreover, they had a shorter length of stay at the ICU than those of the historical group (p = 0.02).
CONCLUSIONS: Peripartum ICU admissions are increasing in frequency. As maternal characteristics are changing, adequate risk stratification with multidisciplinary care are essential, and access to intermediate care units would be preferable for patients with short-term admission.

PMID: 28101669 [PubMed - indexed for MEDLINE]

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Cerebrovascular response to maternal hyperoxygenation in fetuses with hypoplastic left heart syndrome depends on gestational age and baseline cerebrovascular resistance.

Ultrasound Obstet Gynecol. 2018 Oct;52(4):473-478

Authors: Szwast A, Putt M, Gaynor JW, Licht DJ, Rychik J

OBJECTIVES: Compared with normal fetuses, fetuses with hypoplastic left heart syndrome (HLHS) have smaller brain volumes and are at higher risk of brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce the risk of brain injury in these fetuses. This study investigated whether gestational age and baseline cerebrovascular resistance affect the response to MH in fetuses with HLHS.
METHODS: The study population comprised 43 fetuses with HLHS or HLHS variant referred for fetal echocardiography between January 2004 and September 2008. Middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed between 20 and 41 weeks' gestation at baseline in room air (RA) and after 10 min of MH. Z-scores of MCA-PI were generated. A mixed-effects model was used to determine whether change in MCA-PI depends upon gestational age and baseline MCA-PI.
RESULTS: In RA and following MH, MCA-PI demonstrated a curvilinear relationship with gestational age in fetuses with HLHS, peaking at around 28 weeks and then falling more steeply near term. MCA-PI Z-score declined in a linear manner, such that it was 1.4 SD below that in normal fetuses at 38 weeks. Increase in MCA-PI Z-score after MH was first seen at ≥ 28 weeks. A baseline MCA-PI Z-score ≤ -0.96 was predictive of an increase in cerebrovascular resistance in response to MH.
CONCLUSION: In fetuses with HLHS, MCA-PI first increases in response to MH at ≥ 28 weeks' gestation. A baseline MCA-PI Z-score ≤ -0.96 predicts an increase in cerebrovascular resistance in response to MH. These results may have implications for clinical trials utilizing MH as a neuroprotective agent. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

PMID: 28976608 [PubMed - indexed for MEDLINE]

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Psychological interventions for people affected by childhood-onset heart disease: A systematic review.

Health Psychol. 2019 Feb;38(2):151-161

Authors: Tesson S, Butow PN, Sholler GF, Sharpe L, Kovacs AH, Kasparian NA

OBJECTIVE: Guidelines recommend psychological intervention for children, adolescents, and adults with childhood-onset heart disease and their families, yet a comprehensive review of interventions is lacking. We aimed to determine the efficacy of psychological interventions for this population.
METHOD: We searched 6 electronic databases until August, 2017 for English-language, controlled trials of psychological interventions for children, adolescents, or adults with congenital heart disease, inherited arrhythmias, or cardiomyopathies, or their family members. Outcomes of interest included: anxiety, depression, psychological stress and distress, health-related quality of life, coping and adjustment, developmental outcomes, physical health, and parent and family outcomes.
RESULTS: Of 7,660 identified articles, 11 articles reporting on 9 unique interventions met inclusion criteria. Four interventions included adolescents or adults with congenital heart disease, 2 of which also included individuals with cardiomyopathies, valvular heart conditions, or inherited arrhythmias. Five interventions targeted parents, predominantly mothers, of children with congenital heart disease. Clinical and methodological diversity was observed across trials. Parent-focused interventions demonstrated some improvements in maternal mental health, including anxiety and worry, coping, and family functioning. Evidence for the efficacy of interventions for adolescents and adults was limited. Most trials (8/9) were at "high" or "serious" risk of bias.
CONCLUSIONS: Despite an established evidence-base for psychological interventions in other chronic illness populations, evidence of efficacy for children and adults with childhood-onset heart disease and their families was limited. Interventions using conceptual frameworks tested in methodologically robust trials are needed to enhance the provision of mental health care for people of all ages with childhood-onset heart disease. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

PMID: 30652913 [PubMed - indexed for MEDLINE]

Cardiac auscultation: normal and abnormal.

Br J Hosp Med (Lond). 2019 Feb 02;80(2):C28-C31

Authors: Warriner D, Michaels J, Morris PD

PMID: 30747003 [PubMed - in process]

Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

Catheter Cardiovasc Interv. 2019 Feb 12;:

Authors: Miranda WR, Hagler DJ, Reeder GS, Warnes CA, Connolly HM, Egbe AC, Taggart NW

OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.
BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited.
METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP).
RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.
CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

PMID: 30747481 [PubMed - as supplied by publisher]

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Tell me once, tell me soon: parents' preferences for clinical genetics services for congenital heart disease.

Genet Med. 2018 11;20(11):1387-1395

Authors: Kasparian NA, De Abreu Lourenco R, Winlaw DS, Sholler GF, Viney R, Kirk EPE

PURPOSE: As the molecular basis of congenital heart disease (CHD) comes into sharper focus, cardiac genetics services are likely to play an increasingly important role. This study aimed to identify parents' preferences for, and willingness to participate in, clinical genetics services for CHD.
METHODS: A discrete choice experiment was developed to assess parents' preferences for pediatric cardiogenetics services based on four attributes: appointment format, health professionals involved, waiting time, and information format. Data were analyzed using a mixed logit model.
RESULTS: One hundred parents with a living child diagnosed with CHD requiring surgical intervention between 2000 and 2009 completed the discrete choice experiment. Parents expressed a clear preference for cardiac genetics services featuring (i) a single appointment, (ii) the presence of a clinical geneticist and a genetic counselor, (iii) both verbal (oral) and Web-based information about CHD and genetics, and (iv) availability of an appointment within 2 weeks. If offered such conditions, 93% of respondents indicated that they would attend. The choice of service was most strongly influenced by the presence of both a clinical geneticist and a genetic counselor.
CONCLUSION: Parents of children with CHD favor a single, timely genetics appointment with both a geneticist and a genetic counselor present. If appointments offered match these preferences, uptake is likely to be high.

PMID: 29493584 [PubMed - indexed for MEDLINE]

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Not So Incidental: Pheochromocytoma in an Adult with Unrepaired Cyanotic Heart Disease.

Eur J Case Rep Intern Med. 2018;5(10):000959

Authors: Adunuri N, Mrkobrada M

Pheochromocytoma, a rare neuroendocrine tumour, is often encountered in the general internal medicine clinic as an adrenal incidentaloma. Even rarer is its presence in adult cyanotic heart disease, although there are a few documented reports of this association in various paediatric populations, with chronic hypoxia being the likely driving force. Here we present the case of a 38-year-old adult with unrepaired complex cyanotic congenital heart disease with biochemically proven pheochromocytoma presenting as an adrenal incidentaloma to a general internal medicine clinic.
LEARNING POINTS: There is an association between pheochromocytoma and congenital cyanotic heart disease.It is important to maintain clinical vigilance for laboratory testing for pheochromocytoma in patients with cyanotic heart disease who are seen at general internal medicine clinics.

PMID: 30755984 [PubMed]

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Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Card Electrophysiol Clin. 2017 06;9(2):xv-xvi

Authors: Balaji S, Mandapati R, Shivkumar K

PMID: 28457248 [PubMed - indexed for MEDLINE]

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Pregnancy, Marfan syndrome, and type-B aortic dissection.

BJOG. 2018 03;125(4):494

Authors: Johnson MR, Roos Hesselink JW

PMID: 28600893 [PubMed - indexed for MEDLINE]