Echocardiographic assessment of cardiovascular involvements in children with cystic fibrosis.

BACKGROUND: Over the past four decades, numerous case reports and clinical studies have highlighted the presence of heart disease in individuals with cystic fibrosis. Given the limited information in this field and the imperative to identify early changes during childhood, our study aims to explore cardiac dysfunction in patients with cystic fibrosis using echocardiography. METHODS: In this case-control study, we examined echocardiographic findings from thirty-three patients with cystic fibrosis and sixty healthy children. Demographic information for both groups was recorded, and the disease severity in patients was assessed using the Schawachman criterion. M-mode, Doppler flow velocity, and Tissue Doppler Imaging echocardiography were performed for all participants, with subsequent data analysis using SPSS 24. RESULTS: Our study encompassed thirty-three CF patients and sixty healthy children. The estimated pulmonary artery blood pressure (systolic and mean) in patients with cystic fibrosis was significantly higher than in the control group (P < 0.05). Additionally, the mean trans-tricuspid peak early to late diastolic flow velocity (E/A) was significantly lower in the case group than the control group (P < 0.05), along with a significantly lower mean tricuspid valve deceleration time (DT) (P < 0.05). Similarly, the mean TAPSE in the case group was notably lower than in the control group (P < 0.05). No significant difference in Mean left ventricular Ejection Fraction (EF) and Fractional Shortening (FS) existed between the two groups (P > 0.05). Furthermore, Trans-mitral peak early to late diastolic flow velocity (E/A) in the case group was significantly lower than in the control group (P < 0.05), and the mean mitral valve DT in the case group was also significantly lower (P < 0.05). CONCLUSION: Our study findings indicate the presence of some degree of right ventricular dysfunction in children with cystic fibrosis. This finding may have implications for the development or modification of clinical guidelines for managing cystic fibrosis in children. Further investigations are recommended to elucidate the underlying mechanisms and contributing factors, providing valuable insights for clinical management.

Comparison of QT dispersion before and after PDA device closure in pediatrics.

BACKGROUND: Numerous studies have shown that QT dispersion (QTd) can be a suitable criterion for risk assessment of arrhythmia in patients with congenital heart disease. Pulmonary arterial hypertension (PAH) increases the risk of cardiac arrhythmia by changing ventricular repolarization homogeneity. In this study, we assessed QTd changes after PDA device closure and the effect of PAH on these changes. METHODS: Between October 2018 and March 2021, 97 patients (48 males; 49 females; mean age 31.36 ± 4.26 months; range 3 months to 14 years) who satisfied the primary inclusion criteria and did not meet the exclusion criteria and underwent PDA device closure intervention were included in the study. Echocardiography was performed before the procedure. QT corrected (QTc), and QTd and PR intervals were measured according to the patients' standard 12-lead ECGs in two periods, preoperative (1 day) and after (3 months). RESULTS: In the general group, QTc and QTd decreased significantly after PDA closure. Based on our classification of the patients in two groups of high PAP and normal PAP, the three parameters QTc, QTd, and PR interval were assessed separately in the two groups. All three parameters decreased significantly in the normal PAP and high PAP groups. CONCLUSIONS: However, a left-to-right shunt through the patent ductus arteriosus can affect ventricular repolarization; this effect seems to be particularly more significant when there is pulmonary hypertension.

Pulmonary Artery Sling Associated with Stridor from Early Infancy: A Case Report.

Background: Pulmonary artery sling is a rare condition in which the left pulmonary artery anomalously originates from a normally positioned right pulmonary artery. The left pulmonary artery arises anterior to the right main bronchus, courses between the trachea and esophagus then enters the left hilum. Respiratory symptoms such as wheezing, stridor, cough, and dysphasia are common in this anomaly. Case presentation: We describe a 16-month-old male infant presenting recurrent cough, stridor, and wheezing from early infancy. He underwent computed tomography angiography, bronchoscopy, and transthoracic echocardiography, confirming the left pulmonary artery sling diagnosis. Surgical correction of pulmonary artery sling was successfully performed as a new anastomosis between the main pulmonary artery and the left pulmonary artery, as well as tracheoplasty. The infant was discharged without any complications. Follow-up after two years revealed no respiratory symptoms and feeding difficulty. Conclusion: In the presence of chronic cough, stridor, recurrent wheezing, and other prolonged respiratory symptoms, investigation for possible detection of pulmonary artery sling is recommended.

Comparison of the Effect of Nifedipine Alone and the Combination of Nifedipine and Sildenafil in Delaying Preterm Labor: A Randomized Clinical Trial.

Objective: Recently, sildenafil as a drug effective in relaxing smooth muscles can be used as an adjunct to delay the onset of uterine contractions and therefore the occurrence of preterm labor. The aim of this study was to evaluate the effect of nifedipine combination with sildenafil on preterm delivery compared with nifedipine alone. Materials and methods: This randomized double-blinded clinical trial was performed on pregnant women with a gestational age of 26-34 weeks with singleton pregnancy and symptoms of preterm delivery. The mothers were randomly assigned into two groups receiving nifedipine plus sildenafil or those receiving nifedipine alone. The time of delivery, maternal and neonatal complications were compared between the two groups. Results: Mothers who received the combination therapy experienced significantly lower preterm delivery within 72 hours of intervention compared to nifedipine alone (4.5% versus 27.3%, p = 0.002). The rate of delivery during the first 7 days after discharge was 7.6% and 31.8% in nifedipine plus sildenafil and nifedipine alone, respectively (P = 0.001). The prevalence of neonatal respiratory distress syndrome (RDS) as well as mean birth weight was higher in the nifedipine group alone. Treatment protocol with nifedipine and sildenafil compared with nifedipine alone was associated with a significant increase in preterm delivery delay (beta =-5.819, p = 0.001). Conclusion: The use of sildenafil in addition to nifedipine causes more delay in delivery in cases of preterm labor, followed by lower risk for RDS, reduces neonatal intensive care unit (NICU) admission, and preserves neonatal birth weight.

A Rare Case of Cardiac Hydatid Disease without Liver and Lungs Involvement.

Hydatid disease is a parasitic infection caused by Echinococcus granulosus. Cardiac involvement is rare especially without liver and lungs tissue involvement. We describe a 12-year-old male patient referred to Mofid Children's Hospital, Tehran, Iran in Jul 2020 due to chronic pericardial effusion and suspected tuberculosis infection from Afghanistan. Echocardiography revealed a cystic lesion in the interventricular septum. Thoracic and abdominal computed tomography showed no similar cystic lesion in the lungs and liver. The patient underwent open-heart surgery for cystectomy and medical treatment with albendazole. Histological examination confirmed hydatid cyst diagnosis. The patient was discharged in good condition and oral albendazole was continued.

Acute Lymphoblastic Leukemia with Eosinophilia and Strongyloides stercoralis Hyperinfection.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Bone pain is an important symptom that can be severe. Eosinophilia without any other abnormal laboratory findings is rare in ALL. Strongyloides stercoralis in ALL causes disseminated fatal disease. CASE PRESENTATION: This 9-year-old girl presented with bone pain in lumbar region. Bone pain was the only symptom. The patient didn't have organomegaly. The BM samples were studied by flow cytometry, which showed pre-B cell ALL. Larva of Strongyloides stercoralis was found in fecal examination. Plain chest x ray showed bilateral para-cardiac infiltration. Strongyloidiasis was treated before starting chemotherapy. After two days treatment with Mebendazol the patient developed cough, dyspnea, respiratory distress and fever. The treatment changed to Ivermectin for 2 days. Chemotherapy started five days after diagnosis of leukemia. CONCLUSION: The patient complained merely of bone pain in lumbar region without any other signs and symptoms. Peripheral blood smear showed eosinophilia without any other abnormality. Stool examination showed Strongyloides stercoralis larvae. We suggest that all patients diagnosed as ALL in tropical and subtropical regions should be evaluated for parasitic infection especially with Strongyloides stercoralis.