The Nakata index and McGoon ratio: correlation with the severity of pulmonary regurgitation after the repair of paediatric tetralogy of Fallot.

BACKGROUND: Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. RESULTS: When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. CONCLUSIONS: This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot.

Impact of pulmonary valve replacement on left and right ventricular function using strain analysis, in children with repaired tetralogy of Fallot.

BACKGROUND: In repaired Tetralogy of Fallot (rTOF), pulmonary regurgitation and resulting right ventricular (RV) and left ventricular (LV) dysfunction are associated with adverse clinical outcomes. We performed an echocardiographic assessment of LV and RV function using Global Longitudinal Strain (GLS) and conventional echo method prior to and following Pulmonary Valvular Replacement (PVR) to help inform proper timing of operation. RESULTS: A total of 30 rTOF patients (12.17 ± 2.5 years, 70% male) were included. Regarding to LV function, the study revealed a significant reverse correlation between LV GLS (absolute value) and early (mean = 10.4 days) and late (mean = 7.4 months) postop LVEF. Paired T-Test showed significant difference between GLS of LV and RV before and late after operation (op), however, without significant changes early postop. Late postop significant improvements occurred in other conventional echo indices of LV and RV function as well. There was also a significant correlation between echo-measured LVEF & Fraction Area Change (RV FAC) and MRI-derived LVEF & RVEF, respectively. CONCLUSION: In this cross-sectional study in rTOF patients, RV and LV GLS as well as conventional echocardiographic indices regarding LV and RV function improved significantly after 6 months (mean = 7.4mo) following PVR.

The effect of fasting on spirometry indices and respiratory symptoms in asthmatic patients.

Introduction: Ramadan can alter the course of diseases by changing nutrition patterns, sleep habits, and medication-taking schedules. There are some concerns that patients with asthma may be affected by these alterations during Ramadan and experience deterioration of their symptoms. This study aimed to investigate the effect of fasting in Ramadan on the severity of the disease and spirometric parameters in patients with asthma. Methods: An overall 120 patients with moderate to severe asthma were investigated during Ramadan and categorized into two groups of fasting (60 cases) and non-fasting (60 cases) groups. Patients underwent spirometry before and after Ramadan and asthma control status was also assessed. The parameters measured in spirometry were compared in each group before and after Ramadan and also between the two groups. Results: Spirometric measurements including forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), peak expiratory flow (PEF), and FEV1/FVC were not significantly different before and after Ramadan in both groups of fasting and non-fasting patients. Furthermore, there was no significant difference between the two groups in terms of these spirometric parameters changes from baseline. Nevertheless, FEV1 change in the fasting group was significantly higher than that in the non-fasting group (1.46±5.37 vs. -0.13±3.08, respectively; P=0.040). Conclusion: The results of this study demonstrated that fasting has no significant effect on the severity of asthma and spirometric findings in patients with moderate to severe asthma. Therefore, fasting during Ramadan can be considered safe for patients with asthma.

Prevalence of Structural Heart Diseases Detected by Handheld Echocardiographic Device in School-Age Children in Iran: The SHED LIGHT Study.

Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD. Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population. Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics. Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly. Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening.

Pubertal status of children with congenital heart disease.

BACKGROUND: CHD influences many aspects of life in affected individuals. Puberty, a major aspect of development, is a concern for patients and families. OBJECTIVES: We investigated pubertal status in children and adolescents with CHD. METHODS: Patients with CHD aged 6-18 were enrolled. Cardiac diagnoses were confirmed using history, examination, and paraclinical tools including echocardiography. An endocrinologist determined pubertal stages, and the second Tanner stages for pubarche (P2), thelarche (B2), and gonadarche (G2) were considered as the pubertal onset. A study with a large sample size on pubertal onset in a normal population was used for comparison. RESULTS: Totally, 451 patients (228 girls and 223 boys) at a median (10th-90th percentile) age of 10.79 (8.02-14.28) years for the girls and 10.72 (8.05-14.03) years for the boys were enrolled. The median (10th-90th percentile) ages at B2 and P2 in the girls with CHD were 10.77 (9.55-12.68) and 10.53 (9.39-12.28) years, respectively, which were higher than the median ages of 9.74 (8.23-11.94) and 10.49 (8.86-12.17) years in the normal girls.The median (10th-90th percentile) ages at G2 and P2 in the boys with CHD were 11.04 (8.85-13.23) and 11.88 (9.78-13.46) years, correspondingly, which were higher than the median ages of 9.01 (6.00-11.84) and 10.34 (6.84-13.10) years in the normal boys. CONCLUSIONS: Pubertal onset could be delayed in children with CHD when compared with the normal population.

Percutaneous Balloon Pulmonary Valvuloplasty of Critical Pulmonary Stenosis and severe pulmonary stenosis in Neonates and Early Infancy: A Challenge in the Cyanotic.

Introduction: Pulmonary stenosis with an intact ventricular septum (PS-IVS) is one of the common causes of cyanotic heart disease in neonates with diverse morphologies as well as management and treatment protocols. The aim of this study was to evaluate short and midterm results of balloon pulmonary valvuloplasty (BPV) for this disorder. Methods: Between 2012 and 2016, Totally 45 neonates and infants under 6 months old were evaluated.The patients had a minimum right-to-left ventricular pressure ratio of 1, right-to-left shunting at the patent foramen ovale or atrial septal defect level, and tricuspid valve Z-scores higher than -4. Results: Immediately after the procedure, the right ventricular pressure dropped to the normal values in 8 (20%) patients. The immediate procedural success rate was seen in 42 (93.3%) cases: the right-to-left ventricular pressure ratio dropped to below 50% or the level of O2 saturation rose above 75%. Of three cases unresponsive to BPV, two of them underwent patent ductus arteriosus (PDA) stenting and one procedural death occurred. At 6 months' follow-up, of 42 patients, this pressure was still with in the normal range in 36 (80%) infants, while it had returned to high values in 9 (20%) patients and necessitated repeat valvuloplasty. After BPV, severe pulmonary valve regurgitation was observed in14.2% patients; the condition was more common when high-profile noncompliant balloons were used. Conclusion: Balloon pulmonary valvuloplasty in infants with PS-IVS confers acceptable results insofar as it improves echocardiographic parameters and hemodynamic changes at short- and midterm followups.Balloon selection with sizes more than 1.2 of the diameter of the pulmonary valve annulus and the use of noncompliant high-pressure balloons results in higher degrees of pulmonary regurgitation.

A forgotten diagnosis in right heart failure: A case report and literature review.

Carcinoid heart disease is a well-known complication of carcinoid syndrome that affects morbidity and mortality. Carcinoid heart disease may be asymptomatic in the early stages; therefore, patients with carcinoid syndrome should be screened to prevent misdiagnosis.

Pocket-sized echocardiography for screening structural heart disease: diagnostic accuracy and cost-effectiveness for population-based studies.

BACKGROUND: The standard transthoracic echocardiography has some limitations in emergent and community-based situations. The emergence of pocket-sized ultrasound has led to influential advancements. METHODS: In this prospective study, in the hospital-based phase, children with suspected structural heart diseases were enrolled. In the school-based phase, healthy children were randomly selected from six schools. All individuals were examined by experienced operators using both the standard and the pocket-sized echocardiography. RESULTS: A total of 73 individuals with a mean age of 9.9 ± 3.2 years in the hospital-based cohort and 143 individuals with a mean age of 12.8 ± 2.9 years in the school-based cohort were examined. The agreements between the standard and the pocket-sized echocardiography were good or excellent for major CHDs in both cohorts (κ statistics > 0.61). Among valvular pathologies, agreements for tricuspid and pulmonary valves' regurgitation were moderate among school-based cohorts (0.56 [95% confidence interval 0.12-1] and 0.6 [95% confidence interval 0.28-0.91], respectively). The agreements for tricuspid and pulmonary valves' regurgitation were excellent (>0.9) among hospital-based population. Other values for valvular findings were good or excellent. The overall sensitivity and specificity were 87.5% (95% confidence interval 47.3-99.7) and 93.8% (95% confidence interval 85-98.3) among the hospital-based individuals, respectively, and those were 88% (95% confidence interval 77.8-94.7) and 68.4% (95% confidence interval 56.7-78.6) among the school-based individuals, respectively. The cost of examination was reduced by approximately 70% for an individual using the pocket-sized device. CONCLUSIONS: When interpreted by experienced operators, the pocket-sized echocardiography can be used as screening tool among school-aged population.

Assessment of normal hemodynamic profile of mechanical pulmonary prosthesis by doppler echocardiography: a prospective cross-sectional study.

OBJECTIVES: Very few reports have described the Doppler-derived echocardiographic parameters for mechanical pulmonary valve prosthesis (MPVP). This study aims to describe the normal Doppler hemodynamic profile of MPVP using Doppler echocardiography. METHODS: The current prospective, single center observational study enrolled 108 patients who underwent pulmonary valve replacement (PVR) surgery for the first time and had a normally functioning prosthesis post-operation. The hemodynamic performance of MPVPs, considering flow dependent and flow independent parameters, was evaluated at two follow-up points, at week one and week four post-operation. All assessments were conducted by an experienced echocardiographer. RESULTS: The mean age (±SD) of the participants was 26.4 (±8.98). Tetralogy of Fallot (ToF) was the most common underlying disease leading to PVR, with a prevalence of 88%. At first week post-operation, measurement of indices reported the following values (±SD): peak pressure gradient (PPG): 18.51(±7.64) mm Hg; mean pressure gradient (MPG): 10.88(±5.62) mm Hg; peak velocity (PV): 1.97(±0.43)m/s; doppler velocity index (DVI): 0.61(±18); pulmonary velocity acceleration time (PVAT): 87.35(±15.16) ms; effective orifice area (EOA): 2.98(±1.02) cm2;and effective orifice area to body surface area ratio (EOA/ BSA): 1.81(±0.62) cm2/m2. Comparing these measurements with those obtained from the second follow-up (at week four post-op) failed to hold significant difference in all values except for PVAT, which had increased from its primary value (p = 0.038). Also, right ventricular (RV) function showed significant improvement throughout the follow up period. CONCLUSION: The findings of this study help strengthen the previously scarce data pool and better establish the normal values for Doppler hemodynamics in mechanical pulmonary prosthesis.

Left Atrial Drainage of the Right Superior Vena Cava: A Case Report.

An isolated right superior vena cava (RSVC) draining into the left atrium represents a very rare congenital malformation, especially in the absence of a partial anomalous pulmonary venous return. This condition leads to hypoxemia, cyanosis, and clubbing without any other signs of heart defects. We describe an 8-year-old girl, who was referred to our hospital due to unexplained cyanosis. Segmental approach in transthoracic echocardiography showed left atrial drainage of the RSVC, which was subsequently confirmed by contrast echocardiography and angiography. Surgical repair via trans-section and anastomosis of the superior vena cava to the right atrium was performed to prevent the complications of right-to-left shunting and cyanosis. During a 4-year follow-up, the patient remained in very good clinical status and her serial echocardiography was normal except for very mild left atrial and left ventricular enlargement.

Evaluation of NPP1 as a Novel Biomarker of Coronary Artery Disease: A Pilot Study in Human Beings.

Purpose: Coronary artery calcification (CAC) is utilized as an important tool for global risk assessment of cardiovascular events in individuals with intermediate risk. Ecto phosphodiesterase/nucleotide phosphohydrolase-1(ENPP1) converts extracellular nucleotides into inorganic pyrophosphate and it is a key regulator of tissue calcification that adjusts calcification in tissues like vascular smooth muscle cells. The main purpose of this clinical study was to find out the correlation between ENPP1 serum concentration and CAC in human for the first time. Methods: In this study 83 patients (16 diabetic patients and 67 non-diabetic patients) with coronary artery disease who fulfilled inclusion and exclusion criteria, entered the study. For all patients a questionnaire consisting demographic data and traditional cardiovascular risk factors were completed. Computed tomography (CT)-Angiography was carried out to determine coronary artery calcium score and enzyme-linked immunosorbent assay (ELISA) method was used for measuring ENPP1 serum concentrations. Results: There was a reverse significant correlation between ENPP1 serum concentration and total CAC score and also CAC of right coronary artery (RCA) (P<0.05) in non-diabetic patients. Conclusion: On the basis of our results, ENPP1 serum concentration may be a suitable biomarker for coronary artery disease at least in non-diabetic patients. However, more studies with higher sample size are necessary for its confirmation.

Management of Cardiovascular Disorders in Patients with Noonan Syndrome: A Case Report.

The Noonan syndrome is a rare disorder, one of whose major complications is cardiovascular involvement. A wide spectrum of congenital heart diseases has been observed in this syndrome. The most common cardiac disorder is pulmonary valve stenosis, which has a progressive nature. Hypertrophic cardiomyopathy is less common, but its morbidity and mortality rates are high. We herein introduce a 12-year-old boy with the typical findings of the Noonan syndrome. His symptoms began from infancy, and there was a gradual exacerbation in his respiratory and cardiac manifestations with age. The cardiac involvement included right ventricular outflow tract and pulmonary valve stenosis, hypertrophic cardiomyopathy, and subaortic valve stenosis. Due to the progressive course of the disease, surgical repair was done. Although the patient had a difficult postoperative period, his general condition improved and he was discharged. At 3 months' follow-up, his symptoms showed improvement. Additionally, there was a reduction in the echocardiographic parameters of the outflow tract stenosis gradient as well as a significant improvement in the cardiac hemodynamic indices.

Aortic runoff as a sign of intracranial arteriovenous malformation: report of two cases.

BACKGROUND: Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion. CASE PRESENTATION: We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranial arteriovenous malformation and misdiagnosis of congenital heart disease. CONCLUSION: Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation and had the major role in early diagnosis.

Diagnostic errors in echocardiography: review of five interesting pediatric cases.

Echocardiography is considered the primary diagnostic tool for congenital heart diseases. The pediatric echocardiography diagnostic errors, consisting of false positive or discrepant diagnoses, picked up within a 2.5-year period in our pediatric echocardiography laboratory are presented herein. In this case report, the factors contributing to the diagnostic errors are categorized as cognitive such as misidentification/misinterpretation of findings and distraction by other diagnoses; procedural or conditional such as incomplete examination of anatomy/physiology and poor imaging conditions; and finally communicational or informational such as lacking or misleading patient's history and incorrect requisition.The quality of diagnostic pediatric echocardiography can be improved if the operator has sufficient knowledge about the normal growth and development of children, different types of congenital heart defects, and principles of ultrasound physics.

Intraluminal ascending aorta fibroma.

Primary cardiac tumors are quite rare, especially in the pediatric age group, and their atypical presentations often prevent a timely diagnosis. Most primary cardiac tumors in the pediatric age group are benign. Fibromas are generally reported as the second most common primary cardiac tumors in the pediatric age group. These neoplasms are often intramural and involve the left ventricular free wall or the interventricular septum. Although benign, fibromas may become life-threatening by causing arrhythmias or obstruction to the blood flow. A case of supravalvular intraluminal ascending aorta fibroma in a 23-month-old girl, presenting with syncope, is described here; the location is rare and the presentation atypical for this type of tumor. Transesophageal echocardiography helped us to evaluate the anatomic details of the tumor and plan surgery.