Background:Most of the hypogammaglobulinemic patients have a clinical history in favor of allergic respiratory disease. Nevertheless, in these patients the importance and prevalence of atopic disorders have not been completely explained.Objectives:This study was aimed to evaluate atopic manifestations (dermatitis, allergic rhinitis and asthma) and pulmonary function in patients with hypogammaglobulinemia.Patients and Methods:We used the international study of asthma and allergies in childhood (ISAAC) questionnaire in forty-five patients diagnosed with hypogammaglobulinemia and spirometry was done in 41 patients older than 5 years.Results:Spirometry results were normal in 21 (51%), and showed obstructive in 15 (37%) and restrictive pattern in 5 (12%) of the 41 patients who were evaluated. By the end of the study, asthma was diagnosed in nine (20%) patients and other atopies (rhinitis and dermatitis) identified in 10 (22%), and four (9%), respectively.Conclusions:Atopic conditions should be investigated in the hypogammaglobulinemic patients and the prevalence in these patients may be higher than in normal population. Also, it is recommended to perform a pulmonary function test as a routine procedure in patients with hypogammaglobulinemia and atopy should be assessed in these patients.

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BackgroundLate complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan.MethodsIn a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication.ResultsIn patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 ± 0.14 and 0.59 ± 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 ± 0.26, LPA PI = 0.98 ± 0.27) and in normal individuals (RPA PI = 1.59 ± 0.12, LPA PI = 1.64 ± 0.17) for both branches (p = 0.000).ConclusionUsing a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.

Context: Tetralogy of Fallot (TOF) is one of the most important congenital heart diseases that its prognosis can be improved by surgery. However, Right Ventricular (RV) function may be disrupted because of pulmonary regurgitation and other preoperative and intraoperative factors. Access to a simple and reproducible diagnostic index of RV function is important. Echocardiographic index of Right Ventricular Myocardial Performance (RVMPI) has been used as a simple and noninvasive method for the assessment of RV function. Evidence Acquisition: This article review was conducted utilizing PubMed, Web of Science, Scopus and Google Scholar databases with the keywords of "Right Ventricle (RV)", "Myocardial Performance Index (MPI)", and "Tetralogy of Fallot (TOF) repair or correction". Results: RVMPI as a non-geometric echocardiographic index was compared with other echocardiographic indices, QRS duration in ECG, Cardiac Magnetic Resonance (CMR) findings, and exercise capacity in the pediatric and adult studies. Conclusions: This narrative review suggested that RVMPI, especially the tissue Doppler-derived has been used as a useful index of RV function in the follow-up of the repaired TOF patients. However, more research, including systematic reviews are necessary to determine the potential implication of RVMPI in the assessment of RV dysfunction.