Left ventricle hypertrophy and re-modeling in children with essential hypertension: does the race matter?

BACKGROUND: This is the first study to report on the impact of race on differences in the prevalence of echocardiographic left ventricular hypertrophy and left ventricular adaptation at the time of diagnosis of essential hypertension in children. METHODS: This cross-sectional, single-centre study included patients aged 3-18 years who had newly diagnosed essential hypertension. Echocardiography was used to assess left ventricular mass index and left ventricular relative wall thickness. An left ventricular mass index > the 95th percentile for age and gender, and an left ventricular relative wall thickness > 0.42, were used to diagnose left ventricular hypertrophy and concentric adaptation. Various echocardiographic parameters were compared between African Americans and Caucasians. RESULTS: The study included 422 patients (289 African Americans and 133 Caucasians) diagnosed with essential hypertension at a median age of 14.6 (interquartile range; 12.1-16.3) years. Eighty-eight patients (20.9%) had left ventricular hypertrophy. There was no statistically significant difference in the prevalence of left ventricular hypertrophy between African Americans and Caucasians (22.5% versus 17.3%, p=0.22). The median left ventricular relative wall thickness was 0.35 (0.29-0.43), and 114 patients (27.0%) had an left ventricular relative wall thickness > 0.42. The presence of an left ventricular relative wall thickness > 0.42 was significantly higher among African Americans compared to Caucasians (30.1% versus 20.3%, p = 0.04). The African American race was a strong predictor for an left ventricular relative wall thickness > 0.42 (odds ratio 1.7, p = 0.04), but not for left ventricular mass index > the 95th percentile (p = 0.22). Overweight/obesity was a strong predictor for an left ventricular mass index > the 95th percentile. CONCLUSIONS: There was no difference in the prevalence of left ventricular hypertrophy in children with essential hypertension of different races. Obesity, rather than being African American, is associated with left ventricular hypertrophy.

A Unique High-Output Cardiac Hypertrophy Phenotype Arising From Low Systemic Vascular Resistance in Cantu Syndrome.

Background Cardiomegaly caused by left ventricular hypertrophy is a risk factor for development of congestive heart failure, classically associated with decreased systolic and/or diastolic ventricular function. Less attention has been given to the phenotype of left ventricular hypertrophy with enhanced ventricular function and increased cardiac output, which is potentially associated with high-output heart failure. Lack of recognition may pose diagnostic ambiguity and management complexities. Methods and Results We sought to systematically characterize high-output cardiac hypertrophy in subjects with Cantu syndrome (CS), caused by gain-of-function variants in ABCC9, which encodes cardiovascular KATP (ATP-sensitive potassium) channel subunits. We studied the cardiovascular phenotype longitudinally in 31 subjects with CS with confirmed ABCC9 variants (median [interquartile range] age 8 years [3-32 years], body mass index 19.9 [16.5-22.9], 16 male subjects). Subjects with CS presented with significant left ventricular hypertrophy (left ventricular mass index 86.7 [57.7-103.0] g/m2 in CS, n=30; 26.6 [24.1-32.8] g/m2 in controls, n=17; P<0.0001) and low blood pressure (systolic 94.5 [90-103] mm Hg in CS, n=17; 109 [98-115] mm Hg in controls, n=17; P=0.0301; diastolic 60 [56-66] mm Hg in CS, n=17; 69 [65-72] mm Hg in control, n=17; P=0.0063). Most (21/31) subjects with CS exhibited eccentric hypertrophy with normal left ventricular wall thickness. Congestive heart failure symptoms were evident in 4 of the 5 subjects with CS aged >40 years on long-term follow-up. Conclusions The data define the natural history of high-output cardiac hypertrophy resulting from decreased systemic vascular resistance in subjects with CS, a defining population for long-term consequences of high-output hypertrophy caused by low systemic vascular resistance, and the potential for progression to high-output heart failure.

Ross Syndrome Presenting as Heat Exhaustion: A Report of Two Cases.

Ross syndrome is a rare clinical disorder of sweating associated with tonic pupil and areflexia. There are very few case reports of Ross syndrome in dermatology literature, most presenting with patchy hyperhidrosis. Here, we report two isolated cases who had presented to the emergency department with heat exhaustion. Multidisciplinary evaluations of the first case revealed focal anhidrosis, patchy hyperhidrosis, postural hypotension, absent deep tendon reflex, and tonic pupil while the second case had similar features except for postural hypotension, prompting the diagnosis of Ross syndrome. Presentation of these two patients highlights the importance of a high index of suspicion of dysautonomic disorder, interdisciplinary workup of a case of patchy anhidrosis, or hyperhidrosis, which may get missed in busy outpatient department (OPD) visit.

Successful Treatment of Refractory Chronic Bullous Disease of Childhood with Rituximab.

Chronic bullous disease of childhood is a rare subepidermal bullous disease with a hallmark of linear IgA deposition along basement membrane zone seen on direct immunofluorescence. We report a case of a 2-year-old male child, who had recurrent and multiple bullous eruptions over body and he was not responding to conventional therapy. He had earlier developed a drug reaction to dapsone which is considered the drug of choice for this condition. We report successful management of this case with injection rituximab which is a chimeric monoclonal antibody against CD20, which is primarily found on the surface of B cells.

Device-detected nonsustained ventricular tachycardia in adult congenital heart disease without tetralogy of fallot.

OBJECTIVES: To evaluate any association between non-sustained ventricular tachycardia (NSVT) detected by intra-cardiac device and clinical outcomes in repaired adult congenital heart disease (ACHD) without tetralogy of Fallot (TOF). BACKGROUND: NSVT portends a higher risk of serious ventricular tachyarrhythmia in TOF. However its clinical significance when incidentally detected by implantable cardiac device is not well elucidated in non-TOF ACHD cohort. METHODS: We performed a single center, retrospective, longitudinal follow-up study in repaired ACHD (≥18 years) patients without TOF who hosted a pacemaker or automatic implantable cardiac defibrillator (AICD). The cohort was divided based on presence/absence of device detected NSVT. The primary end-point was a composite of sustained ventricular tachycardia (VT), ventricular fibrillation (VF), or sudden cardiac death (SCD). RESULTS: One hundred fifty eight patients (male 56.3%, median [IQR] age of 35 [28-43] years at last follow-up] with longitudinal post-implant follow-up duration of 8 (5-12) years were included. NSVT was detected in 52 (33%) patients. The primary composite end-point was more frequent in NSVT group [11.5% vs. 2.8%; p = .04]. Patients with NSVT were (i) older at the time of initial implant (age 25 vs. 18 years, p = .011) and more frequently demonstrated (ii) systemic ventricular dysfunction (44% vs. 26%; p = .015), as well as (iii) history of ventriculotomy (38% vs. 21%; p = .017). CONCLUSIONS: In our repaired ACHD cohort, we noted a significant association between device-detected-NSVT and the primary composite end-point of sustained VT/VF or SCD. Systemic ventricular dysfunction and history of ventriculotomy were more frequent in the NSVT group and likely constituted the clinical milieu.

Use of 27-gauge Tuberculin Syringe for Trichloroacetic Acid Chemical Reconstruction of Skin Scars (TCA-CROSS).

Chemical Reconstruction of Skin Scars (CROSS) is a minimally invasive, safe, and cost effective technique for the management of ice pick acne scars that are otherwise generally resistant to treatment. 100% trichloro acetic acid (TCA) is usually applied using toothpicks and the end point is white discoloration of skin. The concentrated caustic agent quite often spills onto surrounding normal skin leading to superficial burns and post-inflammatory pigmentation. Use of 27-gauge tuberculin syringe gives a much more controlled access to apply TCA leading to better cosmetic outcome and fewer adverse effects.

Cardiac Performance in the First Year of Age Among Preterm Infants Fed Maternal Breast Milk.

Importance: There is a beneficial association between human breast milk exposure in the neonatal period and cardiac mechanics in adults who were born preterm. It is unknown whether this benefit is apparent in infants in the first year of age. Objective: To test the hypothesis that higher consumption of mother's own milk in preterm infants is associated with enhanced cardiac performance during the first year of age. Design, Setting, and Participants: This cross-sectional study of cardiac and nutritional data at an academic medical center included 80 individuals born preterm and 100 individuals in the control group born full-term. All births were between 2011 and 2013. Two-dimensional echocardiograms were performed at 32 weeks' and 36 weeks' postmenstrual age and at 1 year's corrected age in individuals born preterm and at 1 month and 1 year of age in individuals born full-term. Statistical analysis was performed from January to May 2021. Exposures: Consumption of mother's own milk. Main Outcomes and Measures: Main study outcomes included echocardiography measures of right and left ventricle longitudinal strain (function), left ventricle mass index and right ventricular areas (morphology), and pulmonary vascular resistance (pulmonary hemodynamics) at age 1 year. Results: Of 180 infants included in the study, 97 (54%) were Black infants and 89 (49%) were female infants. Among the 80 infants born in the preterm cohort, 43 (54%) were female infants and 43 (54%) were Black infants. The median gestational age at birth of the preterm infants was 27.0 weeks (interquartile range, 26.0-28.0 weeks) and the median birth weight was 960 g (interquartile range, 800-1138). For each week of exposure to mother's own milk, preterm infants had greater magnitudes of right ventricular strain (eg, right longitudinal strain: ß, 0.021; 95% CI, 0.002-0.041; P < .001) and left ventricular strain (eg, left longitudinal strain: ß, 0.065; 95% CI, 0.049-0.080; P = .01), larger right ventricle areas (eg, systolic area: ß, 0.026; 95% CI, 0.011-0.042; P = .009), larger left ventricle mass index (ß, 0.045; 95% CI, 0.024-0.073; P = .003), and decreased pulmonary vascular resistance (eg, pulmonary artery acceleration time: ß, 0.041; 95% CI, 0.018-0.063; P < .001) at 1 year's corrected age, even after accounting for gestational age and common neonatal morbidities. Cardiac values approached those seen in controls born full-term with increased mother's own milk exposure. There were no differences in any of the cardiac indices at 32 weeks' postmenstrual age, but with each week of exposure, right ventricle function (eg, right longitudinal strain: ß, 0.016; 95% CI, 0.002-0.031; P < .001) was greater and pulmonary pressured (eg, pulmonary artery acceleration time: ß, 0.0032; 95% CI, 0.0013-0.0062; P < .001). Conclusions and Relevance: This study found that preterm infants with higher consumption of mother's own milk had enhanced cardiac performance at age 1 year, suggesting that mother's own milk consumption may play a dynamic modulator role on cardiac mechanics in preterm-born infants and help in normalization of the preterm cardiac phenotype.

Left Ventricle Phenotyping Utilizing Tissue Doppler Imaging in Premature Infants with Varying Severity of Bronchopulmonary Dysplasia.

Bronchopulmonary dysplasia (BPD) is characterized by alveolar-capillary simplification and is associated with pulmonary hypertension (PH) in preterm infants. The contribution of left ventricle (LV) disease towards this severe BPD-PH phenotype is not well established. We aimed to describe the longitudinal trajectory of the LV function as measured by tissue Doppler imaging (TDI) and its association with BPD-PH. We retrospectively assessed prospectively acquired clinical and echocardiographic data from 77 preterm infants born between 2011 and 2013. We characterized the LV function by measuring systolic and diastolic myocardial velocities (s', e', a'), isovolumetric relaxation time (IVRT), and myocardial performance index with TDI at three time periods from 32 and 36 weeks, postmenstrual age through one year of age. We also measured post systolic motion (PSM), a marker of myocardial dysfunction that results from asynchronous movement of the ventricular walls, and not previously described in preterm infants. Patients were stratified into groups according to BPD severity and the presence of PH and compared over time. Conventional TDI measures of the LV function were similar between groups, but the septal PSM was significantly prolonged over the first year of age in patients with BPD-PH. PSM provides a novel objective way to assess the hemodynamic impact of lung and pulmonary vascular disease severity on LV function in preterm infants with BPD and PH.

A retrospective, 5-year, clinicoepidemiological study of severe cutaneous adverse reactions (SCARs).

BACKGROUND: A severe cutaneous adverse reaction (SCAR) is a rare, clinically heterogeneous, life-threatening phenomenon that results in serious skin damage, systemic complications, and significant morbidity or mortality comprising of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS-TEN overlap, acute generalized exanthematous pustulosis (AGEP), exfoliative dermatitis, and drug rash with eosinophilia and systemic symptoms (DRESS). MATERIALS AND METHODS: A retrospective study was conducted on all cases of SCARs admitted in a tertiary care referral hospital from January 2015 to December 2019. Clinical and epidemiological details were retrieved from the records of patients as per predesigned proforma. The data collected were analyzed and statistically evaluated. RESULTS AND ANALYSIS: A total of 142 patients (67 males, 75 females) with SCARs, constituting 0.08% of total hospital admission and 0.027% of total dermatology outpatient department (OPD), were studied. Age group ranged from 2 to 61 years, with the mean age of 33.6 years (SD = 17.43). Most patients belonged to SJS-TEN complex (75 cases; SJS 48, SJS-TEN 18, TEN nine) followed by exfoliative dermatitis (27 cases/19%), AGEP (26 cases/18.3%), and DRESS (14 cases/9.8%). Anticonvulsants were most commonly implicated (22%) followed by antibiotics (20%), nonsteroidal anti-inflammatory drugs (NSAIDs) (17%), and ayurvedic (7%). There was only single mortality in a DRESS patient. CONCLUSION: SCAR has considerable disease burden with marginal female preponderance in SJS/TEN and exfoliative dermatitis and can involve even the pediatric population. Anticonvulsants followed by antibiotics, NSAIDs, and ayurvedic medicines are common groups known to cause SCARs. To date, there are no definitive recommendations regarding their optimal treatment regimen, hence early diagnosis, prompt withdrawal of culprit drug, high standard of nursing care, and interdisciplinary consultations are vital steps to avoid disease progression and restore health.

Maturational patterns of left ventricular rotational mechanics in pre-term infants through 1 year of age.

BACKGROUND: Pre-mature birth impacts left ventricular development, predisposing this population to long-term cardiovascular risk. The aims of this study were to investigate maturational changes in rotational properties from the neonatal period through 1 year of age and to discern the impact of cardiopulmonary complications of pre-maturity on these measures. METHODS: Pre-term infants (<29 weeks at birth, n = 117) were prospectively enrolled and followed to 1-year corrected age. Left ventricular basal and apical rotation, twist, and torsion were measured by two-dimensional speckle-tracking echocardiography and analysed at 32 and 36 weeks post-menstrual age and 1-year corrected age. A mixed random effects model with repeated measures analysis was used to compare rotational mechanics over time. Torsion was compared in infants with and without complications of cardiopulmonary diseases of pre-maturity, specifically bronchopulmonary dysplasia, pulmonary hypertension, and patent ductus arteriosus. RESULTS: Torsion decreased from 32 weeks post-menstrual age to 1-year corrected age in all pre-term infants (p < 0.001). The decline from 32 to 36 weeks post-menstrual age was more pronounced in infants with cardiopulmonary complications, but was similar to healthy pre-term infants from 36 weeks post-menstrual age to 1-year corrected age. The decline was due to directional and magnitude changes in apical rotation over time (p < 0.05). CONCLUSION: This study tracks maturational patterns of rotational mechanics in pre-term infants and reveals torsion declines from the neonatal period through 1 year. Cardiopulmonary diseases of pre-maturity may negatively impact rotational mechanics during the neonatal period, but the myocardium recovers by 1-year corrected age.

Coronary-cameral fistula with double-chambered right ventricle: appearance on cardiac magnetic resonance imaging and 3D printed anatomic modeling.

The present case illustrates cardiac magnetic resonance imaging (MRI) and three-dimensional (3D) printed anatomic model findings of a coronary-cameral fistula (CCF) and double-chambered right ventricle (DCRV). A pregnant woman presented with palpitations and near syncope. A non-contrast cardiac MRI showed CCF connecting to a DCRV. Post-delivery, the patient had a contrast-enhanced MRI and 3D printed anatomic model to better evaluate her aberrant anatomy.