Pulsatility Index in Different Modifications of Fontan Palliation: An Echocardiographic Assessment.

BACKGROUND: Adding pulsation to the Fontan circulation might change the fate of patients palliated by this procedure. Our aim was to compare the pulsatility index (PI) of the pulmonary artery (PA) between the various modifications of Fontan palliation. METHODS: Doppler-derived PI was measured in PA branches of a cohort of 28 patients palliated by 6 modifications of Fontan procedure. A group of normal individuals was included for comparison. RESULTS: Atriopulmonary connection (APC) group had the highest PA branches PI and statistically was close to the PI of the normal individuals (right pulmonary artery [RPA] PI of 1.58 vs. 1.63; p = 0.99 and left pulmonary artery [LPA] PI of 1.54 vs. 1.68; p = 0.46, respectively). The lowest PA branches PI was seen in the group of extracardiac total cavopulmonary connection (RPA PI of 0.62 and LPA PI of 0.65). Other 4 modifications including the extracardiac conduit with oversewn pulmonary valve, extracardiac conduit with preserved adjusted antegrade flow, extracardiac conduit from inferior vena cava onto the rudimentary right ventricle and lateral tunnel had a mean "RPA and LPA" PI of "1.19 and 1.17", "1.16 and 1.11", "1.13 and 1.11", "0.82 and 0.84", respectively. The modified Dunnett's post hoc test has shown a significant statistical decline in PI of all modifications compared to the normal individuals except for the APC group. CONCLUSIONS: Fontan palliated patients in different groups of surgical modification showed a spectrum of Doppler-derived PI with the highest amounts belong to the groups of pulsatile Fontan.

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.

Echocardiographic Assessment of Pulmonary Arteries Pulsatility Index in Fontan Circulation.

BACKGROUND: Late 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. METHODS: In 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. RESULTS: In 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). CONCLUSION: Using 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.

Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment.

The fundamental role of pulmonary vascular resistance in the Fontan circulation is obvious. Medications decreasing this resistance may have an impact on the fate of this population. Hence, we assessed noninvasively the effect of oral sildenafil on the ventriculo-arterial coupling in patients with Fontan circulation. In a single-center, prospective case series study, 23 patients with fenestrated extracardiac total cavopulmonary connection age 12-31 years were enrolled in this study. Clinical characteristics and echocardiographic examination were performed before and after a 1 week course of sildenafil at 0.5 mg/kg every 8 h. Sildenafil had no effect on heart rate and blood pressure. However, oxygen saturation was significantly increased with sildenafil (87.6 ± 4.3 vs. 90.1 ± 3.6; P < 0.0001). The calculated noninvasive ventricular end-systolic elastance (Ees) was greater after sildenafil compared with the pre-sildenafil values (1.59 ± 0.17 vs. 1.72 ± 0.27 mm Hg/ml; P = 0.001). Moreover, significant decreases in arterial elastance (Ea) (1.62 ± 0.53 vs. 1.36 ± 0.43 mm Hg/ml; P < 0.0001), ventricular end-diastolic elastance (Eed) (0.05 ± 0.021 vs. 0.04 ± 0.013; P = 0.002), and, finally, ventriculo-arterial coupling index (0.99 ± 0.26 vs. 0.76 ± 0.15; P < 0.0001) were found after sildenafil administration. The intolerable side effects that led to stopping the sildenafil occurred only in one (4 %) patient. Sildenafil has increased ventricular systolic elastance and improved ventriculo-arterial coupling in patients palliated with Fontan circulation. Short-term sildenafil was well tolerated in most of the patients with only minor side effects.

Right ventricular myocardial tissue velocities, myocardial performance index, and tricuspid annular plane systolic excursion in totally corrected tetralogy of fallot patients.

BACKGROUND: Longer survival after the total repair of the Tetralogy of Fallot increases the importance of late complications such as right ventricular dysfunction. This is a prospective study of the right ventricular function in totally corrected Tetralogy of Fallot patients versus healthy children. METHODS: Thirty-two healthy children were prospectively compared with 30 totally corrected Tetralogy of Fallot patients. Right ventricular myocardial tissue velocities, right ventricular myocardial performance index, and tricuspid annular plane systolic excursion were investigated as well as the presence and severity of pulmonary regurgitation. RESULTS: The two groups were age-and sex-matched. Mean systolic peak velocity (Sa) and tricuspid annular plane systolic excursion were significantly decreased, while myocardial performance index and early to late diastolic velocity (Ea/Aa) were significantly increased in the Tetralogy of Fallot patients. Early diastolic velocity (Ea) showed no significant difference between the two groups. Sa correlated significantly with tricuspid annular plane systolic excursion in both the normal children and totally corrected Tetralogy of Fallot patients. Myocardial performance index was significantly higher in the patients with moderate to severe pulmonary regurgitation than in those with mild regurgitation. However, there was no significant correlation between this index and right ventricular myocardial tissue velocities. CONCLUSION: In this study, systolic right ventricular function indices (Sa and tricuspid annular plane systolic excursion) were impaired in the totally corrected Tetralogy of Fallot patients. Myocardial performance index was affected by the severity of pulmonary regurgitation.

Avoiding cardiopulmonary bypass in extracardiac cavopulmonary connection: does it really matter?

OBJECTIVES: We examined the effect of avoiding cardiopulmonary bypass on the early outcome variables after fenestrated extracardiac total cavopulmonary connection. METHODS: Between May 2001 and January 2009, 102 patients with univentricular heart physiology underwent fenestrated extracardiac total cavopulmonary connection. Patients were divided into one of 2 groups: the cardiopulmonary bypass (n = 48) group and the no cardiopulmonary bypass (n = 54) group. In both groups there were patients with primary and staged fenestrated extracardiac total cavopulmonary connection. Duration of mechanical ventilation, pleural effusion, hemodynamic status, incidence of arrhythmia, and mortality were compared between the 2 groups. RESULTS: Both groups were matched, except for more cases of tricuspid atresia in the no cardiopulmonary bypass group (P = .014) compared with other diagnostic morphologies and higher preoperative hemoglobin levels in the no cardiopulmonary bypass group (P = .01). Avoiding cardiopulmonary bypass did not reveal any significant effect on postoperative outcomes. A cardiopulmonary bypass time of more than 120 minutes caused not only a meaningful increase in the mean of mechanical ventilation duration (35 +/- 9.6 vs 13 +/- 2.1 hours, P = .026) but also increased the incidence of mechanical ventilation for more than 12 hours (P = .04). Bypass time of more than 120 minutes did not have influence on any other postoperative variables. CONCLUSION: Avoiding cardiopulmonary bypass in fenestrated extracardiac total cavopulmonary connection had no direct effect on the early outcome variables.

Aortic arch aneurysm in a child: the role of multislice computed tomography.

A 10-year-old girl came to our cardiac clinic complaining of chest pain. She had a history of aortic arch reconstruction 9 years ago. On chest X-ray, a calcified dilated aortic arch was found. Additionally, using a multislice computed tomography scan, the dimensions of the aneurysm were better illustrated. During the reoperation, the aneurysm was resected.

The effect of ventricular septal defect enlargement on the outcome of Rastelli or Rastelli-type repair.

OBJECTIVE: Our purpose was to evaluate the effect of ventricular septal defect enlargement on the early and late morbidity and mortality of patients undergoing Rastelli or Rastelli-type operations. METHODS: A total of 49 patients who underwent Rastelli or Rastelli-type operations between 1991 and 2007 were included in a retrospective follow-up study. Patients were divided into 2 groups: group A had ventricular septal defect enlargement, and group B did not have ventricular septal defect enlargement for comparison. Risk factor analysis for early or late death included patient-related and procedure-related variables, with failure, arrhythmia, and atrioventricular block as outcome parameters. RESULTS: Median age and weight at the time of the operation were 6 years (range, 3 months-22 years) and 17 kg (range, 7-48 kg), respectively. The ventricular septal defect was enlarged in 28 (57%) patients. Ventricular septal defect enlargement showed a significant statistical relation with late ventricular dysfunction, arrhythmia, and residual ventricular septal defect (P = .023, P = .047, and P = .01, respectively, log-rank test). No relation was found between ventricular septal defect enlargement and permanent pacemaker implantation (P = .73, log-rank test). Furthermore, enlargement of the ventricular septal defect did not show any significant effect on the rate of early mortality (P = .69, Cox regression). Kaplan-Meier estimated survival for patients with ventricular septal defect enlargement was 74% at 5 years and 65% at 10 years. Freedom from late death in the group without ventricular septal defect enlargement was 100% at 5 and 10 years and 83% at 15 years. At a median follow-up of 4 years (range, 6 months-16 years), there were 12 late-onset deaths: 11 in group A (n = 28) and 1 in group B (n = 21). Ventricular septal defect enlargement greatly increased the risk of late death (P = .009, Cox regression). CONCLUSIONS: Septal resection in patients undergoing Rastelli or Rastelli-type operations has a substantial effect on late morbidity and is a predictive factor for long-term mortality.

Cardiac and great vessel involvement in "Behcet's disease".

Behcet's disease is a multisystem disorder and classified as "vasculitic syndrome with a wide variety of clinical manifestations." Cardiac involvement is very rare but can occur with different presentations including: pericarditis, cardiomyopathy, endocarditis, endomyocardial fibrosis, intracavitary thrombosis, and coronary artery disease. Great vessel involvement is more common. Recurrent Phlebitis, commonly involving large vessels (superior vena cava, inferior vena cava, hepatic veins) and cerebral veins are the sole presentation in this regard. Arterial involvement is expressed by aneurysm or pseudoaneurysmal formation. Due to the wide variety of cardiovascular manifestations and the resulting high mortality, cardiac surgeons should be familiar with this disease. In this paper we review the articles and introduce our four cases presenting with aneurysm of ascending aorta with free aortic insufficiency, aneurysm of descending aorta, pulmonary artery aneurysm, and pseudoaneurysm of aortic arch.

Congenital right atrial aneurysm in an infant.

A 4-month-old infant with right atrial aneurysm, presenting with recurrent episodes of lower respiratory tract infection is described. There was no history of supraventricular tachyarrhythmias or embolic events. Transthorasic echocardiography definitely showed an aneurysmal sac (3.7 A-4.2 cm) originating from the right atrium with compressing effect on left side cardiac structures. Surgical excision of aneurysm allowed removal of mass effect and clinical improvement.