Transcatheter Ventricular Septal Defect Closure with Lifetech™ Konar-MF Occluder in Infants Under 10 kg with Only Using Venous Access.

Transcatheter closure of VSD remains a complex procedure in infants with technical challenges and carries the risk of significant complications, due to its complex anatomical morphology and closed proximity to the atrioventricular valves and the conduction system. In this article, we presented transcatheter VSD closure in infants under 10 kg using the Lifetech Konar-MF device via only venous route without TEE guidance and arterial access. Between January 2021 and May 2023, a total of 34 patients weighing less than 10 kg who underwent transcatheter VSD closure antegradely with Lifetech™ Konar-Multifunctional (MF) occluder were included in the study. The mean age of the patients was 8.1 (3.5-35) months. Average weight was 6.5 kg (range 4.5-10 kg). VSD was perimembranous in 27 patients (79.4%). Successful device placement was achieved in all 34 patients. However, device embolization occurred in three patients. One of the patients was successfully implanted with a one size larger device, the surgical closure was performed other two cases. TR occurred in seven patients (20.6%) after releasing devices. None of the patients developed complete heart block. Right bundle branch block developed in two patients. Residual shunt was observed in 9 patients (six small, two moderate, and one large). During follow-up, residual shunt disappeared in six of these patients and only mild residual shunt remained in the other four patients which have not required any further intervention. Transcatheter closure of VSD with Lifetech Konar-MF device is safe and effective in infants less than 10 kg via only venous access with a high success rate and low complication rate. In these patients, transcatheter VSD closure can be performed by excluding the risk of complications that may occur due to AV loop formation, arterial intervention, endotracheal intubation and TEE use.

Early outcomes of the treatment of aortic coarctation with BeGraft aortic stent in children and young adults.

We report our experience and early outcomes of using the BeGraft aortic stent in children, adolescents, and young adults. BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany) requires a smaller long sheath compared to other covered stents, and it has a low profile and adequate radial power. With these features, it can overcome some limitations in the treatment of coarctation, especially in children. This is a single centre retrospective analysis of 11 implanted BeGraft aortic stents in coarctation of the aorta between July 2020 and November 2021. The eleven stents were successfully implanted in 11 patients (10 males). The median age of the patients was 13.7 years (interquartile range 12-16 years), and the median weight was 43 kg (interquartile range 35-62 kg). In five patients, after the stents were opened completely by the first balloon, they were exchanged with a Z-MED II™ balloon, 1-3 mm larger in diameter, and the stents were redilated. The median catheter-derived systolic peak-to-peak pressure gradient was 23 mm Hg (interquartile range 16-37 mmHg) before the procedure and 3 mm Hg (interquartile range 1-5 mm Hg) after the procedure. Except for the partial femoral artery thrombosis in two patients, no other procedural complications were observed in our study. The median follow-up duration was 5 months (interquartile range 2-12 months). During follow-up, only one patient (9%) had stent narrowing that required dilation. Our initial results and short-term follow-up showed that the BeGraft aortic stent implantation and redilation can be performed effectively, safely, and successfully in the treatment of coarctation of the aorta.

Idiopathic Hypereosinophilic Syndrome Associated With Pulmonary Hypertension in an Infant.

Hypereosinophilic syndrome (HES) is a very rare disease during childhood. It involves the different organs like skin, gastrointestinal system, heart and lungs, besides pulmonary hypertension (PHT) is a very rare morbidity of HES that may cause life-threatening complications. PHT improves with the treatment of hypereosinophilia, without the need for pulmonary vasodilator therapy. Here, we present a case of PHT developed after recovery of pulmonary infiltration in an infant with idiopathic HES. We revealed that pulmonary pressure returned to normal range in parallel with the decrease in eosinophil count with steroid treatment.

Transitioning from Radiofrequency Ablation to Cryoablation for Treatment of Pediatric Atrioventricular Nodal Reentrant Tachycardia: A Single Tertiary Center Experience.

Catheter ablation of the slow pathway is the recommended treatment for atrioventricular nodal reentrant tachycardia (AVNRT) in children. Both radiofrequency ablation (RF) and cryoablation (CA) have been used for this purpose. In this report, we describe our experience during the transition period from RF to CA for the treatment of pediatric AVNRT. Between January 2012 and August 2021, a retrospective evaluation was conducted of the clinical features, procedural outcomes, and follow-ups of pediatric AVNRT patients who underwent catheter ablation at a pediatric electrophysiology center. The catheter ablation outcomes of 89 pediatric AVNRT patients were evaluated: 29 patients were ablated using RF (RF group) and 60 patients were ablated using CA (CA group). No significant difference was found between the groups in terms of gender, age, weight, and success and recurrence rates. The procedure duration and total lesion numbers were statistically significantly lower in the RF group compared with the CA group (86.67 ± 45.8 and 156.1 ± 37.7 min; p = 0.01, 4 [3-6] and p < 0.01, 8 [7-9] lesions, respectively). Catheter ablation was successful in all patients. There were no permanent complete atrioventricular blocks in both groups. A total of six patients (6.8%) developed recurrences. The cryoablation of pediatric AVNRT is a safe and effective procedure with comparable acute and mid-term follow-up success rates compared with RF, even during a period of transition from RF to CA.

Echocardiographic Findings and Correlation with Laboratory Values in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19.

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p < 0.001), E/A ratio was significantly lower, and averaged E/e' ratio was significantly higher (median 1.5 vs. 1.8, p < 0.05; 8.9 vs. 6.3, p < 0.001 respectively) in patients compared to control. Patients were divided into 2 groups according to 3D LV ejection fraction (LVEF) on the echo-1: Group 1; LVEF < 55%, 26 patients, and group 2; LVEF ≥ 55%, 24 patients. E/e' ratio was significantly higher in group 1 than group 2 and control at discharge (median 7.4 vs. 6.9, p = 0.005; 7.4 vs. 6.3, p < 0.001 respectively). Coronary ectasia was detected in 2 patients (z score: 2.53, 2.6 in the right coronary artery), and resolved at discharge. Compared with group 2, group 1 had significantly higher troponin-I (median 658 vs. 65 ng/L; p < 0.001), NT-pro BNP (median 14,233 vs. 1824 ng/L; p = 0.001), procalcitonin (median 10.9 vs. 2.1 µg/L; p = 0.009), ferritin (median 1234 vs. 308 µg/L; p = 0.003). The most common findings were ventricular systolic dysfunction recovering during hospitalization, and persisting LV diastolic dysfunction in the reduced LVEF group at discharge. Coronary artery involvement was rare in the acute phase of the disease. Also, in MIS-C patients, the correlation between LV systolic dysfunction and markers of inflammation and cardiac biomarkers should be considered.

New parameters on prediction of severity of transient tachypnea of the newborn.

BACKGROUND: Transient tachypnea of the newborn (TTN) is a common clinical problem that often occurs in the first hours of life. Although it is considered to be a benign clinical course, some cases may have severe symptoms and require ventilation support. In this study, we aimed to determine the association between the mean platelet volume (MPV), nucleated red blood cells (NRBCs), right ventricular systolic pressure (RVSP), and the severity of TTN. METHODS: Patients with TTN were divided into two groups according to Silverman score (<7: group 1 [n: 34] and ≥7: Group 2 [n: 30]). The groups were compared in terms of demographic characteristics, hematologic parameters, and RVSP within the first 24 hours after admission. RESULTS: Mean birth weight of the patients was 3033.4 ± 364.1 g and median gestational age was 38 weeks (min-max: 34-42). Patients in Group 2 were found to require higher nasal continuous positive airway pressure (nCPAP) support and longer duration of oxygen treatment (p: 0.001). Patients in Group 2 had significantly higher thrombocyte, absolute NRBCs count, NRBCs/100 WBCs, and RVSP levels (p < 0.05). Hemoglobin and hematocrit levels were found significantly higher in group 1(p < 0.05). In logistic regression analysis, NRBCs/100 WBCs was found to be the most important independent parameter that affects Silverman score at admission (OR: 7.065, CI: 1.258-39.670, p: 0.026). DISCUSSION: This is the first study that investigates the association between NRBCs, RVSP, and severity of TTN. We think that elevated NRBCs and RVSP values are helpful for clinicians in decision making for referral of the patients to a secondary or a tertiary level of NICU and also inform the families about prognosis.

Therapeutic plasma exchange: A potential management strategy for critically ill MIS-C patients in the pediatric intensive care unit.

This study aimed to evaluate the effectiveness and the role of therapeutic plasma exchange (TPE) in treatment of children with severe MIS-C. In addition, we assessed demographic data, clinical features, laboratory abnormalities, underlying conditions, treatments, and outcomes. Patients with severe MIS-C who were admitted to the pediatric intensive care unit (PICU) between September 01 and October 05, 2020 were included in this observational, descriptive, retrospective study. The data collected included the patients' demographic data, presenting symptoms, clinical features, laboratory parameters, diagnostic investigations, and medications. Of 27 children with MIS-C, 63 % were male. The median age of the patients was nine years. Intravenous immunoglobulin and corticosteroids were used for treatment in 100 % of the patients, anakinra in 51.8 %, vasopressors in 85.1 %, noninvasive mechanical ventilation in 25.9 %, and invasive mechanical ventilation in 18.5 %. Ten of the 27 patients (37 %) underwent TPE. In the patients who underwent TPE, the median PELOD score was 21 (IQR: 11-30.25) before TPE and 10 (IQR: 10-11) after TPE (p < 0.001). Moreover, their median left ventricular ejection fraction (LVEF) was 52 % (IQR: 49.25 %-55 %) before TPE and median LVEF was 66.5 (IQR: 58 %-68.5 %) after TPE (p = 0.012). The median number of TPE sessions was three (IQR: 2-4.75). The mortality rate of the patients with severe MIS-C admitted to the PICU was 7.4 %. We suggest that TPE should be considered as a therapeutic option in children with severe MIS-C. Early initiation of TPE followed by immunomodulatory therapy in critically ill children with MIS-C may help improve clinical and laboratory outcomes.

Assessment of Cardiac Arrhythmic Risk in Children With Covid-19 Infection.

Coronavirus disease of 2019 (COVID-19) is a cause of significant morbidity and mortality worldwide. Although COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported. The mechanism of cardiac injury and arrhythmias is unclear. Also, drugs currently used to treat the COVID-19 may prolong the QT interval and may have a proarrhythmic propensity. The study aims to investigate the effects of COVID-19 infection with asymptomatic and mild symptoms on trans-myocardial repolarization parameters in children without treatment. A total of 105 COVID-19 patients were compared with 40 healthy children. The patient and control group data were compared by calculating the QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e, Tp-e dispersion, Tp-e/QT ratio, and Tp-e/QTc ratio on the 12-lead surface electrocardiogram. The mean age was determined as 11.2 ± 0.3 years in the patient group, and 10.8 ± 2.1 years in the control group. In the COVID-19 group, QTd, QTcd, Tp-e, Tp-e dispersion, Tp-e/QT ratio and Tp-e/QTc ratio were statistically higher than the control group. The ventricular repolarization was impaired even in asymptomatic children with COVID-19 infection. These results suggest the need to further assess the long terms risks of prolonged QT dispersion in the setting of COVID-19 infection.

Transcatheter closure of post-operative type 1 Gerbode defect by Amplatzer Duct Occluder 2.

Acquired and congenital left ventricular to right atrial communication is rare, but nowadays, the frequency of the iatrogenic subgroup is increasing. Successful transcatheter closure of these defects with different devices has been reported. Herein, we presented successful closure of left ventricular to right atrial communication with Amplatzer Duct Occluder 2 after attempting to close with a failed Amplatzer Vascular Plug II device in a 7-year-old girl. This report supports that transcatheter closure of iatrogenic Gerbode defect with Amplatzer Duct Occluder 2 device is safe and effective.

Balloon coarctation angioplasty with non-compliant balloon in low-birth-weight premature infants.

Although surgery is the standard treatment for native coarctation in neonates, it carries a high risk of complications. Percutaneous balloon angioplasty may be considered as an alternative treatment. The materials used in the intervention should be selected carefully to reduce complications. We recommended the use of non-compliant balloons in risky babies. They are more effective in the treatment of strick stenosis than compliant balloons.

Midodrine treatment in children with recurrent vasovagal syncope.

BACKGROUND: Vasovagal syncope is the most common cause of syncope in childhood and its treatment is not at a satisfactory level yet. We aimed to investigate patients who were diagnosed with vasovagal syncope, did not benefit from conventional treatment, received midodrine treatment, and to evaluate their response to midodrine treatment. METHODS: Files of 24 patients who were diagnosed with recurrent vasovagal syncope, did not benefit from non-pharmacological treatments, and received midodrine treatment during June 2017-October 2019 were retrospectively analysed. RESULTS: In total, 24 patients received a treatment dose of midodrine at 5 mg/day (2.5 mg BID) included in the study. The mean number of syncope was 5.75 ± 2.67 prior to treatment. Following treatment, the mean number of syncope was 0.42 ± 0.89. It was observed that syncope episodes did not recur in 17 patients, but it recurred in 4 out of 7 patients in the first 3 months of the treatment and did not recur in the following months. The episodes improved in two patients with an increase in the treatment dose, but the syncope episodes continued in only one patient. CONCLUSION: It was concluded that midodrine treatment was effective and safe in adolescents with recurrent vasovagal syncope.

Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary trunk.

Anomalous origin of coronary artery originating from the pulmonary artery in conjunction with the aorticopulmonary window (APW) is a rare but a significant anomaly in the era of congenital cardiac diseases. The occurrence of anomalous origin of the right coronary artery from the pulmonary artery among the associated anomalies is less than 5%. The severity of the clinical condition of these patients depends on the degree of left-right shunt and compromise of the pulmonary blood flow. We report surgical management of a case of a 45-day-old infant with APW, ventricular septal defect, and anomalous origin of coronary artery originated from the pulmonary artery.

Diastolic dysfunction in patients with brucellosis despite the absence of infective endocarditis.

BACKGROUND: Brucellosis is an important systemic infectious disease, especially in developing countries. Every organ and system of the human body can be affected; however, cardiovascular complications of brucellosis are rare. AIM: To assess cardiac functions in patients with acute brucellosis without overt cardiac involvement and to answer the following question: Is there any cardiac dysfunction despite the absence of endocarditis in these patients? METHODS: This cross-sectional study included 67 children with brucellosis and 40 healthy children. We performed a detailed echocardiographic examination in individuals at the beginning of the treatment. Patients with infective endocarditis were excluded from the study. RESULTS: Echocardiography revealed no difference of ejection fraction, mitral and tricuspid annular plane systolic excursion, pulsed-wave Doppler-derived early diastolic peak velocity (E)/late diastolic peak velocity (A) ratios in mitral and tricuspid valves between the two groups. The deceleration time of early mitral inflow was longer in patients with brucellosis. Early diastolic peak velocity of the mitral and tricuspid annuluses obtained by tissue Doppler imaging (Ea) was significantly lower in children with brucellosis. The peak velocity obtained by tissue Doppler imaging during late diastole (Aa), Ea and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall and right ventricle free wall was lower in patients with brucellosis than in the control group. The E/Ea ratio, isovolumic relaxation time, right ventricle and left ventricle myocardial performance indices were higher in patients with brucellosis. CONCLUSION: Patients with acute brucellosis may have diastolic dysfunction without overt cardiac involvement and infective endocarditis.

Pulmonary infections due to brucellosis in childhood.

INTRODUCTION: Brucellosis is widely distributed zoonotic infection. Brucellosis is a multisystemic disease but pulmonary infection due to brucellosis is very rarely reported. MATERIALS AND METHODS: We retrospectively evaluated our pediatric brucellosis cases between February 2014 and December 2015. The brucellosis patients with and without pulmonary infection were compared with each other. RESULT: We detected pulmonary infection in 18 of the 98 brucellosis patients. There was no statistical significant difference between the brucellosis patients with and without a pulmonary infection as regards age, gender, and animal husbandry history. The laboratory findings including the Brucella Coombs agglutination titers were also similar. The most common chest X-ray findings were interstitial infiltration and hilar lymphadenopathy. All of our cases with pulmonary infection recovered with the usual anti-brucella treatment. CONCLUSIONS: Brucellosis may cause a pulmonary infection more often than thought. The prognosis of respiratory brucellosis is good and the classic treatment regimen is appropriate.

Third Generation Ventricular Assist Device: Mid-Term Outcomes of the HeartWare HVAD in Pediatric Patients.

The HeartWare HVAD is a small, third generation continuous flow pump that is intracorporeally placed for support of a failing ventricle in adult patients. This device is small in size when compared to other left ventricular assist devices and can therefore be used in smaller sized pediatric patients. We present our initial experience using the HVAD as a bridge to heart transplantation in the pediatric population. We performed a retrospective, single center, nonrandomized review of 17 pediatric patients who underwent HVAD implantation between June 2013 and March 2016. The primary endpoints evaluated in this study were overall survival to heart transplantation, ongoing device support, or death. In this patient cohort, nine (53%) of 17 patients were male. The median age of the patients was 13.4 ± 3.8 (range 5-17) years. The median body surface area was 1.4 ± 0.4(0.7-2) m2 . Etiologies of heart failure requiring HVAD support were dilated cardiomyopathy (n = 8), myocarditis (n = 5) and noncompaction cardiomyopathy (n = 4). The overall mean length of HVAD support was 254 ± 298 (range 2-804) days. A successful outcome (bridge to transplant and ongoing mechanical support) was achieved in 13 patients (76.5%). Of the 13 patients, nine (69.2%) were bridged to heart transplantation and four continue to receive support (30.7%) and are eligible for transplantation. Post-transplant survival has been 100%, with a mean follow-up of 296 ± 264.5 (range 18-785) days. The most common complication was pump thrombosis (23.5%) in follow-up. Four patients (23.5%) experienced no complications. The HVAD continuous flow ventricular assist device can be safely used to bridge pediatric patients to cardiac transplantation. Favorable outcomes of this device are comparable to the adult population. This analysis demonstrated safe and effective implantation of the HVAD System in a child with a BSA of 0.7 m2 .

Assessment of cardiac functions in infants with cow's milk allergy.

BACKGROUND: Cow's milk allergy is the most common food allergy in children, with rates estimated at 1.9% to 4.9%. Clinical phenotypes of cow's milk allergy are varied and involve 1 or more target organs, with the main targets being the skin, respiratory system, and gastrointestinal tract. To date, no studies have investigated detailed cardiac function in children with cow's milk allergy. The current study aimed to investigate cardiac function in infants with cow's milk allergy. MATERIAL/METHODS: We studied 42 infants with cow's milk allergy and 30 age- and sex-matched healthy subjects. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. RESULTS: There were no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the 2 groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Ea/Aa ratios in the left ventricle posterior wall and right ventricle free wall were lower in patients with cow's milk allergy than in the control group. The E/Ea ratio in the left ventricle, isovolumic relaxation time, deceleration time, and right and left ventricular myocardial performance indices were higher in patients in the study group. CONCLUSIONS: Our study identified reduced early diastolic tissue Doppler velocities in infants with cow's milk allergy.

Impact of patent ductus arteriosus and subsequent therapy with ibuprofen on the release of S-100B and oxidative stress index in preterm infants.

BACKGROUND: Hemodynamically significant patent ductus arteriosus (hsPDA) leads to injury in tissues/organs by reducing perfusion of organs and causing oxidative stress. The purpose of this study was to evaluate the oxidant/antioxidant status in preterm infants with hsPDA by measuring the total antioxidant capacity and total oxidant status and to assess neuronal damage due to oxidant stress related to hsPDA. MATERIAL AND METHODS: This prospective study included 37 low-birth-weight infants with echocardiographically diagnosed hsPDA treated with oral ibuprofen and a control group of 40 infants without PDA. Blood samples were taken from all infants, and than the total antioxidant capacity (TAC), total oxidant status (TOS), and S-100B protein levels were assessed and oxidative stress index was calculated before and after therapy. RESULTS: The mean pre-therapy TOS level and oxidative stress index (OSI) value of the patients with hsPDA were significantly higher, but TAC level was lower than in the control group. There were no statistically significant differences in the mean post-therapy values of TOS, TAC, OSI, and S-100B protein between the two groups. CONCLUSIONS: hsPDA may cause cellular injury by increasing oxidative stress and damaging tissue perfusion; however the brain can compensate for oxidative stress and impaired tissue perfusion through well-developed autoregulation systems to decrease tissue injury.

Assessment of cardiac functions in fetuses of gestational diabetic mothers.

We investigated cardiac function in 67 fetuses of gestational diabetic mothers (FGDMs) and 122 fetuses of healthy mothers between 24 and 36 weeks of gestation. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. Fetal echocardiograms were performed at 24, 28, 32, and 36 weeks of gestation. Glycated hemoglobin (HbA1c) levels were obtained from all pregnant women at 24 weeks of gestation. The mean age of diabetic pregnant women was significantly greater than that of controls. Serum HbA1c values of both groups were within normal limits, but they were significantly greater in the diabetic group (p = 0.003). The increase in peak aortic and pulmonary artery velocities were greater in FGDM (p < 0.001). No pathological interventricular septal (IVS) hypertrophy was observed. There was a significant increase in IVS thickness in FGDM compared with controls, which was more prominent at the end of the third trimester (p < 0.001). During the course of pregnancy, mitral E-wave (p < 0.001), A-wave (p = 0.007), tricuspid E-wave (p < 0.001) and A-wave (p = 0.002) velocities were greater in FGDM. The increases in mitral E/A and tricuspid E/A ratios were lower in FGDM with advancing gestation. The E a-wave (p = 0.02), A a-wave (p = 0.04), and S a-wave (p < 0.001) velocities of the right-ventricular (RV) free wall and the E a (p = 0.02) and A a (p = 0.01) velocities of the left-ventricle (LV) posterior wall were greater in FGDM during the course of pregnancy. The E a/A a ratio of the RV posterior wall was greater in FGDM with advancing gestation (p < 0.03). LV and RV E/E a ratios were similar in both groups. The LV myocardial performance index measured by pulsed-wave Doppler was greater in FGDM (p < 0.001). We detected diastolic dysfunction in FGDM. The data suggest that gestational diabetes mellitus may impair ventricular diastolic functions without causing pathological fetal myocardial hypertrophy. We detected subclinical diastolic dysfunction using both pulsed-wave and tissue Doppler imaging in FGDM.

The effects of pre-pregnancy obesity on fetal cardiac functions.

Obesity is a substantial public health problem with a rapidly increasing prevalence in numerous industrialized nations. The objective of this study was to evaluate the effects of maternal pre-pregnancy obesity on fetal cardiac functions. We studied 55 fetuses of obese mothers and 44 fetuses of healthy mothers at 26-38 weeks of gestation. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. The two groups were similar in terms of maternal age, gravidity, parity, gestational age, estimated birth weight, serum lipids, and systolic-diastolic blood pressure. Fetal heart rate, diameters of the aortic and pulmonary valve annulus, aortic and pulmonary peak systolic velocities, ventricular systolic function, and cardiothoracic ratio were similar in the two groups. Pulsed-wave Doppler-derived E/A ratios in the mitral and tricuspid valves were similar in the two groups. The deceleration time of early mitral inflow was prolonged in the fetuses of the obese mothers. In the interventricular septum, left ventricle posterior wall, and right ventricle free wall, the E a and A a were higher, and E a/A a ratios were significantly lower in the study group than in the control group. The E/E a ratio was higher in the obese group than in the control group. The isovolumic relaxation time and the right and left ventricle myocardial performance indices were higher in the fetuses of the obese mothers than in the fetuses of the healthy mothers. We believe that maternal obesity has an important influence on fetal cardiac diastolic functions.

Evaluation of cardiac functions of patients with benign joint hypermobility syndrome.

We sought to investigate whether echocardiography with tissue Doppler imaging identifies myocardial dysfunction in children with benign joint hypermobility syndrome (BJHS). This cross-sectional study enrolled 75 children with BJHS and 70 healthy children. We performed detailed echocardiography in individuals with BJHS without inherited connective tissue disorders. Any congenital or acquired cardiac disease was excluded by clinical and echocardiographic examination. Both groups were similar in terms of age, sex, and body mass index. The diameter of the aortic annulus and sinus valsalva were wider in patients with BJHS. There was no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the two groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Deceleration time of early mitral inflow was prolonged in patients with BJHS. Mitral and tricuspid annulus Ea velocity were significantly lower in children with BJHS. Ea, Aa, and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall, and right ventricle free wall were lower in patients with BJHS than in the control group. The E/Ea ratio was greater in patients with BJHS than in the control group. Isovolumic relaxation time and right-ventricular (RV) and left-ventricular (LV) myocardial performance indices (MPIs) were greater in patients with BJHS. This study showed the diastolic dysfunction in patients with BJHS. In addition, we detected increased LV and RV MPI. We believe that BJHS may affect proteins of the myocardial cytoskeleton and extracellular matrix.