Correlation between echocardiographic estimation of right atrial pressure and invasive measurement of central venous pressure in postoperative pediatric patients with congenital heart disease: a prospective observational study.

BACKGROUND: Right atrial pressure plays a critical role as a hemodynamic parameter in diagnosing pulmonary hypertension and other cardiac diseases, as well as guiding the treatment and prognosis of various cardiac disorders. If there is no obstruction between the inferior or superior vena cava (SVC) as central veins and the right atrium, the pressures in these veins could be considered equal to the right atrial pressure. This study aimed to examine the correlation between echocardiographic methods for estimating right atrial pressure and invasive measurements of central venous pressure (CVPi) in infants and children with congenital heart disease during the 48 h after cardiac surgery and to establish regression equations for echocardiographic estimation of central venous pressure (CVPe). RESULTS: We prospectively enrolled 43 infants and children, ranging in age from 6 months to 16 years, including 20 males and 23 females. We found a significant correlation between CVPi and the ratio of the maximal diameter of IVC to the maximal diameter of the descending aorta ratio (IVCmax/DAOmax) (r = 0.529, P < 0.001), SVCS/D velocity ratio (SVCS/D) (r = 0.462, P = 0.006), right atrial vertical diameter (RAVD) (r = 0.409, P = 0.01), area (r = 0.384, P = 0.014), and tricuspid valve A wave acceleration rate (TVAAR) (r = 0.315, P = 0.048). Multiple regression analysis yielded an equation for estimating central venous pressure using four parameters related to the IVC, SVC, tricuspid valve, and right atrium. The equation is as follows: estimated CVP = 4.36 + (2.35 × IVCmax/DAOmax) + (1.06 × SVCS/D) + (0.059 × RAVD) + (0.001 × TVAAR). This equation is strongly correlated with CVPi (Pearson r = 0.698, P = 0.002). CONCLUSIONS: The estimation of central venous pressure through a multi-parametric equation that included the ratio of the maximal diameter of the inferior vena cava to the maximal diameter of the descending aorta, the ratio of S to D velocity of the superior vena cava, the vertical diameter of the right atrium, and the acceleration rate of the A wave of the tricuspid valve demonstrated a robust correlation with invasively measured central venous pressure. To assess the accuracy of predicted pressures by this equation, further investigations are required to apply this innovative multi-parametric formula to a prospective population of pediatric patients with congenital heart disease.

Neglected Partial Anomalous Pulmonary Venous Return during Atrial Septal Defect Device Closure.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart malformation in which 1 or more pulmonary veins drain into the systemic venous circulation or directly into the right atrium instead of the left atrium. It may occur alongside other congenital heart defects, including atrial septal defect (ASD). All patients with newly diagnosed ASD must be evaluated thoroughly for the likelihood of PAPVR to select surgical or percutaneous procedures. Here, we describe a 10-year-old girl with PAPVR who underwent percutaneous device closure of her secundum ASD with her PAPVR neglected at 3 years of age. We had to correct the anomalous venous connection by removing the device during an intricate procedure. The typical connection of the pulmonary veins to the left atrium was reported on her postoperative echocardiography 1 day after surgery. The patient was discharged without complications, and her first follow-up visit 7 days after discharge was unremarkable. While the most accurate diagnostic tools for PAPVR are cardiovascular magnetic resonance imaging and computed tomographic angiography, a careful examination of the pulmonary veins during pulmonary angiography or transesophageal echocardiography in children helps identify PAPVR in patients with ASD.

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.

Safety of Aortic Coarctation Treatment in Patients with Turner Syndrome: A Single-Country Case Series and Literature Review.

BACKGROUND: Coarctation of the aorta is a common cardiac disease in Turner syndrome. Evidence indicates that surgery and balloon angioplasty in infants and small children do not have any added risk of mortality or complication in these patients. Stenting in older patients may, however, pose higher risks of arterial wall injury and mortality. METHODS: In this case series, we describe 15 patients with coarctation of the aorta in Turner syndrome: 9 received stenting, 4 underwent surgery, and 2 were treated via balloon angioplasty. RESULTS: Dissection occurred in 2 patients after stenting: 1 in the aorta and the other in the external femoral artery. Both were managed promptly without any mortality or serious damage, one percutaneously and the other surgically. CONCLUSIONS: Awareness of increased risks and preparedness for prompt interventions in case of an acute arterial wall injury are recommended when coarctation stenting is done for a patient with Turner syndrome.

Fresh frozen plasma prime and the level of gammaglobulin after pediatric cardiopulmonary bypass.

Different organ perturbation and multiple complications might occur after cardiopulmonary bypass (CPB). A variety of solutions might be used for pump priming with different advantages and disadvantages. The advantage of fresh frozen plasma (FFP) inclusion in pump prime has been shown in post-CPB coagulation management. Acquired hypogammaglobulinemia is the disadvantage of albumin (ALB) pump prime. Our aim was to assess the impact of FFP prime on the post-pump serum level of immunoglobulin G (IgG) and its subclasses. Fifty-six patients under the age of 5 years old who were scheduled for cardiac surgery on CPB were randomly primed with FFP or ALB. Any innate or acquired immune deficiency was considered as exclusion criteria. The pre-CPB and 24-hour post-CPB collected blood samples were analyzed by the nephelometric method for the plasma level of IgG and its four subclasses. Twenty-two patients (mean age and weight of 13 months and 6.8 kilograms) in the ALB prime group and 26 patients (mean age and weight of 15 months and 8.1 kilograms) in the FFP prime group completed the study. Using paired t-test and repeated measures ANOVA test, patients in the ALB prime group had a significant drop in the post-CPB serum level of total IgG (597±138 mg/dL to 379±179 mg/dL, P value <0.001) and its two subclasses of IgG1 and IgG3. In contrast, there was a slight elevation in the serum level of total IgG (549±207 mg/dL to 630±180 mg/dL, P value =0.008) and its two subclasses of IgG2 and IgG4 in patients who had FFP prime solution. In conclusion, compared to the ALB prime solution, FFP inclusion in prime could hamper the pediatric post-CPB induced hypogammaglobulinemia.

Giant congenital right atrial epithelioid-capillary hemangioma with prolonged QT interval: Case report and practical surgical treatment strategy for primary cardiac tumors in children based on 25-year review of 299 cases.

Cardiac hemangioma is very rare and accounts for 2%-3% of the primary cardiac tumors. Cardiac epitheloid-capillary hemangioma has not been reported in the pediatric population so far. We report the fatal outcome of a preterm neonate with a huge congenital right atrial epitheloid-capillary hemangioma and elevated serum alpha-fetoprotein, associated with prolonged QT interval. We describe the echocardiographic, computed tomographic (CT) imaging, microscopic and immunohistochemical features of the tumor. Complete resection of the tumor was done at operation necessitating extensive reconstruction of atrial walls. Intramural infiltration of this tumor into the surrounding myocardial walls is a challenging characteristic of cardiac hemangioma. The temptation to complete resection should be avoided in the setting of extensive intramural infiltration and entrapment of the tumoral cells into atrial walls, particularly in a preterm neonate. None of the current classifications for hemangioma was inclusive of our case. Based on a concise literature review of nine published classification systems from 1996 to 2017 , we discuss the shortcomings of the current classifications for hemangioma. We also performed a 25-year-review of 299 cases of primary cardiac tumors in neonates and children, from 1993 to May 2018. We suggest a stepwise surgical treatment strategy according to the characteristics of the patient and of the tumor, based on this review. The stepwise strategy includes watchful observation, partial resection, complete resection and cardiac transplantation.

Iranian homograft heart valves: assessment of durability and late outcome.

Durability and the rate of complications of homograft heart valves, adjusted for patient-related contributors and surgical techniques, rely mainly on the quality of allografts which in turn are mirrored in the donor characteristics and most importantly recovery and processing procedures. Aimed to assess the quality, a study was conducted to figure out the durability and late outcome following homograft replacement with valved conduits procured by the Iranian Tissue Bank. Retrospectively, the pre-implantation, perioperative and follow-up data of 400 non-consecutive recipients of cryopreserved heart valves (222 pulmonary and 178 aortic) from 2006 to 2015 were collected and analyzed in terms of variables reflecting late outcome including adverse events and durability. In the context of durability, the event of interest was defined as the need for homograft replacement and homograft-related death. The mean follow-up time (SD) of study entrants (male/female ratio, 1.4) was 49.8 (36.3) months. Median age at the time of implantation was 11 years. Total 10-years mortality was 21 % (84/400), including 66.7 % early (30-days mortality: 56/84) and 33.3 % late (28/84). Overall late complication rate was 2 %. Median survival time was 120 months (95 % CI 83.3-156.6). The pulmonary valves appeared to be more durable (P value <0.001) and survival probabilities in small sized grafts were lower (P value 0.008). One-, five-, and ten-year graft survival was 82, 76 and 73 %, respectively. The evidences suggest that the homografts function satisfactory with low rate of late complications; nevertheless, more emphasis should be given to make long-term durability comparable.

Relation between preoperative mild increased in serum creatinine level and early outcomes after coronary artery bypass grafting.

This study evaluates the effect of preoperative increased level of serum creatinine (Cr) on early outcomes after coronary artery bypass graft surgery (CABG). 1140 patients who underwent CABG in our center were studied. Patients with Cr >2.25 mg/dl or preoperative dialysis and who had off-pump operations were excluded. Group 1 consisted of 892 patients with normal Cr (0.5-1.2 mg/dl) and group 2 consisted of 248 (21.8%) patients with mild increased level of serum Cr (1.3-2.2 mg/dl). Patients in group 1 were younger than group 2. There were more patients with hypertension in group 2, but there were not statistically significant difference between two groups in terms of the frequency of diabetes, smoking, cerebrovascular disease and New York Heart Association (NYHA) class. Left ventricular ejection fraction (LVEF) was lower in group 2. Cardiopulmonary bypass time (CPB) was longer in group 2. Early mortality was 3.2% in group 1 and 8.4% in group 2 (P<0.001). Prolonged ICU stay, low cardiac output, prolonged mechanical ventilation, postoperative atrial fibrillation, postoperative re-exploration and sepsis were more frequent in group 2. Mild increase in serum Cr level preoperatively is a marker of increased early mortality and outcome after CABG.

Ross operation in a neuro-cardio-facial-cutaneous syndrome patient.

Congenital heart diseases are a major part of Costello and cardio-facio-cutaneous syndromes. Subaortic stenosis was reported rarely and Ross operation never in these syndromes.We reported a girl patient whose manifestations were consistent with these syndromes. Distinction between these syndromes was not possible as genetic testing was not carried out. She developed severe neoaortic regurgitation 2.5 years after the Ross operation and died due to the complications of aortic valve replacement.Ross operation may be an unsuitable option in these syndromes due to the possibility of subtle pulmonic valve pathology.

Does combined antegrade-retrograde cardioplegia have any superiority over antegrade cardioplegia?

BACKGROUND: In a prospective randomised clinical study we assessed and compared antegrade vs. combined antegrade-retrograde cardioplegia in patients who underwent elective coronary artery by pass grafting. METHODS: Between March 2006 and January 2007, 87 consecutive patients were randomly divided into two groups. Group A (n=45) received antegrade cold (4 degrees C) blood cardioplegia. Besides antegrade cardioplegia, Group B (n=42) received continuous retrograde cardioplegia passively by gravitational force. The need for cardiac support during and after cardiopulmonary bypass, post-operative morbidity, ICU stay, hospital stay and mortality were compared in two groups. RESULTS: There was no significant difference between the two groups in gender, age and pre-operative ejection fraction. Sixteen patients in Group A (35.5%) and eight patients in Group B (19%) needed inotropic support while weaning off cardiopulmonary bypass (p=0.04). Four patients in Group A (8.9%) and two patients in Group B (4.8%) needed intra-aortic balloon pump (p=0.44) in the ICU. We found no statistically important difference between the two groups in post-operative morbidity and mortality. CONCLUSIONS: Retrograde continuous infusion of cardioplegia by gravitational force combined with antegrade cardioplegia, provides satisfactory myocardial protection and eliminates the need for inotropic support compared with antegrade technique alone.

Oral ascorbic acid in combination with beta-blockers is more effective than beta-blockers alone in the prevention of atrial fibrillation after coronary artery bypass grafting.

Because adrenergic beta antagonists are not sufficient to prevent atrial fibrillation after coronary artery bypass grafting, this prospective, randomized trial was designed to evaluate the effects of ascorbic acid as an adjunct to beta-blockers. Fifty patients formed our ascorbic acid group, and another 50 patients formed our control group. All patients were older than 50 years, were scheduled to undergo coronary artery bypass grafting, and had been treated with beta-blockers for at least 1 week before surgery. The mean age of the population was 60.19+/-7.14 years; 67% of the patients were men. Patients in the ascorbic acid group received 2 g of ascorbic acid on the night before the surgery and 1 g twice daily for 5 days after surgery. Patients in the control group received no ascorbic acid. Patients in both groups continued to receive beta-blockers after surgery. Telemetry monitoring was performed in the intensive care unit, and Holter monitoring was performed for 4 days thereafter. The incidence of postoperative atrial fibrillation was 4% in the ascorbic acid group and 26% in the control group (odds ratio, 0.119; 95% confidence interval, 0.025-0.558, P = 0.002). We conclude that ascorbic acid is effective, in addition to being well-tolerated and relatively safe. Therefore, it can be prescribed as an adjunct to beta-blockers for the prophylaxis of post-bypass atrial fibrillation.

Autologous in vitro expanded mesenchymal stem cell therapy for human old myocardial infarction.

BACKGROUND: Stem cell transplantation after myocardial infarction has been claimed to restore cardiac function. Mesenchymal stem cells attract a lot of attention because of the feasibility of in vivo and ex vivo differentiation to cardiomyocytes and endothelial cells as well as their trophic effect on tissue repair. In this study, we investigated the efficacy of autologous bone marrow derived mesenchymal stem cells in improving heart function in patients with old myocardial infarction. METHODS: Eight patients with old myocardial infarction and proper inclusion criteria were injected with mesenchymal stem cells at the time of coronary artery bypass grafting or percutaneous coronary intervention (test group) and compared with eight matched patients who received the same treatment without mesenchymal stem cell injection (control group). Evaluation of heart function was done by echocardiography plus single-photon emission computed tomography before and six months after the procedure. Serial clinical examination was performed every month through New York Heart Association class. RESULTS: The mean New York Heart Association class and single-photon emission computed tomography scan results decreased significantly in the test group (P=0.000 and 0.002, respectively) and in the control group (P=0.049 and 0.007, respectively) after the procedure at six months follow-up. Left ventricular ejection fraction increased significantly in the test group (P= 0.005) but not in the control group. In comparison between the test and control groups the results of New York Heart Association class assessment and single-photon emission computed tomography demonstrated significant improvement in the test group (P=0.005 and 0.013, respectively). There were no significant differences between the baseline variables in the two groups. CONCLUSION: Transplantation of ex vivo expanded bone marrow derived mesenchymal stem cell in patients with old myocardial infarction is a safe and feasible procedure. These cells improve the cardiac function without serious adverse effects.

The Ross operation: clinical results and echocardiographic findings.

Between November 2001 and September 2004, 80 patients aged 11 to 56 years (mean, 27.6 years) underwent the Ross operation. The mean preoperative New York Heart Association functional class was 2.37 +/- 0.72, and the mean ejection fraction was 52.8% +/- 16%. Aortic involvement included stenosis in 19 (24%) patients, regurgitation in 22 (28%), and both in 39 (49%). Root replacement was the technique used in all cases. The mean hospital stay was 5 days, and 74 patients (93%) were followed up for 4-48 months. Four-year actuarial survival rate was 96.25%. Postoperative echocardiography revealed no pulmonary autograft insufficiency in 50 patients (63%), trivial to mild insufficiency in 22 (28%), moderate insufficiency in 2 (3%), and severe insufficiency in one (1%). Two patients required autograft re-intervention. Postoperative echocardiography of the pulmonary homograft valve showed severe stenosis (peak gradient > 50 mm Hg) in 2 patients, and moderate stenosis (peak gradient 25-50 mm Hg) in one. The mean postoperative left ventricular ejection fraction was 51.4%. The Ross operation can be considered an elegant alternative to prosthetic valves in the treatment of aortic valve diseases in developing countries.

One common polymorphism of cholesteryl ester transfer protein gene in Iranian subjects with and without primary hypertriglyceridemia.

Primary hypertriglyceridemia is considered to be a major risk factor for pancreatitis, atherosclerosis and coronary heart disease. Cholesteryl ester transfer protein gene polymorphisms known to be associated with changes in lipid levels. This study was performed by using polymerase chain reaction and restriction fragment length polymorphisms. Genotype distribution and allelic frequencies of polymorphism were determined and compared in primary hypertriglyceridemic and normotriglyceridemic subjects. The results showed that plasma cholesteryl ester transfer protein activity was significantly higher in primary hypertriglyceridemia than in controls (p = 0.001). In this study all individuals with B2B2 genotype had lower plasma cholesteryl ester transfer protein activity, higher high-density lipoprotein than B1B1 and B1B2 genotypes, whereas triglyceride was significantly decreased in this genotype. The genotype and allelic frequencies for this polymorphism differed significantly between primary hypertriglyceridemic patients and controls (p = 0.014 and p = 0.027, respectively). In both groups, CETP Taq 1B polymorphism (presence of B2 allele) correlated significantly with HDL-C (r = 0.207 and 0.300 in control and patient groups, respectively) and CETP activity (r = -0.193 for controls and r = -0.132 for patients). Taq 1B polymorphism of cholesteryl ester transfer protein gene was associated with changes in lipids profile and plasma cholesteryl ester transfer protein activity in the selected population.

Repair of Ebstein anomaly: early and mid-term results.

BACKGROUND: Ebstein anomaly is the downward displacement and adherence of dysplastic septal and posterior tricuspid leaflets into the right ventricle, thereby dividing the ventricle into a so-called atrialized chamber and a functionally reduced right ventricle. We evaluated the early and mid-term results of primary repair of Ebstein anomaly in adult patients. METHODS: Eight consecutive patients undergoing repair of Ebstein anomaly with Danielson technique at Imam Khomeini Medical Center, Tehran, Iran from January 1997 through July 2004 were evaluated. Functional and echocardiographic parameters were studied both preoperatively and postoperatively, as well as demographic status and adverse events. RESULTS: Hospital mortality occurred in one patient because of right ventricular failure. The average follow-up period was 5.3 +/- 3.4 years (median: 3.8 years). The actuarial survival rate was 85.7 +/- 4.8% at 7 years. During the follow-up, six patients were in New York Heart Association functional class I, and one patient was in class II. None of the patients required reoperation related to their Ebstein repair. One patient demonstrated atrioventricular dissociation perioperatively; however, only one patient required permanent pacemaker insertion later. One patient had minimal (1+) regurgitation, with the jet localized along the anterior part of the ventricular septum. Two patients had residual tricuspid valve insufficiency (2+) on echocardiography. CONCLUSION: Ebstein repair has a good functional and hemodynamic outcome in adult patients.