Left Ventricular Mechanics in Patients with Abnormal Origin of the Left Main Coronary Artery from the Pulmonary Trunk Late after Successful Repair.

OBJECTIVE: Our aim was to evaluate left ventricular (LV) mechanics by using speckle tracking echocardiography (STE) in asymptomatic patients with abnormal origin of the left main coronary artery from the pulmonary trunk (ALCAPA), late after successful repair, in the presence of LV ejection fraction (EF) >50%. METHODS: We studied 30 ALCAPA patients (median age 4 years, range 1-25 years, NYHA class I, LVEF >50%) and 16 healthy age- and sex-matched controls (median age 5 years, range 1-25 years). All underwent standard echocardiographic evaluation and STE. RESULTS: LV dimensions and LVEF (63.6 ± 8.2% vs. 64.1 ± 5.1%, p = 0.826) were not different between patients and controls. Diastolic parameters were significantly abnormal in our patients versus controls (E/e' average: 11.9 ± 5.8 vs. 6.6 ± 3.0, p = 0.0014). Global LV longitudinal strain was significantly lower in ALCAPA patients versus controls (-17.6 ± 3.5% vs. -23.4 ± 3.1%, p < 0.0001). LV torsion (9.1 ± 4.9° vs. 11.9 ± 3.3°, p = 0.046) was significantly impaired in ALCAPA patients. CONCLUSIONS: After successful repair in asymptomatic ALCAPA patients, despite an LVEF >50%, diastolic function, LV longitudinal deformation and LV torsion remain impaired. We suggest including a detailed study of the diastolic function and cardiac mechanics in the clinical follow-up of these patients to identify the subgroup of patients at higher risk.

Simultaneous aortic and mitral valve replacement in children: time-related outcomes and risk factors.

BACKGROUND AND AIM OF THE STUDY: Valve replacement in children is problematic, and associated with high anticoagulation-related complications and increased reoperation requirements. Multiple valve replacement may further increase morbidity and worsen outcome. The results are reported of combined aortic valve replacement (AVR) and mitral valve replacement (MVR) in children. METHODS: The medical records of children who underwent simultaneous AVR and MVR between 1984 and 2004 were reviewed, and the short-term and long-term results and variables affecting outcomes explored. The mean duration of follow up was 9.7 +/- 6.6 years. RESULTS: A total of 84 patients (62 males, 22 females; mean age 15.0 +/- 2.2 years) was identified. The underlying pathology was mainly rheumatic (94%) and endocarditis (4%). Among the patients, 21 (25%) had undergone a prior cardiac surgery. The implanted valves were either mechanical (n = 71) or bioprosthetic (n = 13). The average aortic and mitral valve sizes were 22 mm and 29 mm, respectively. In total, 21 patients had concomitant cardiac surgery, most commonly tricuspid valve repair (n = 18). The mean cardiopulmonary bypass time and ischemic time were 142 +/- 47 min and 107 +/- 33 min, respectively. Survival at 30 days and at one year was 96% and 94%, respectively. The overall 15-year survival was 78% (bioprosthesis 92% versus mechanical 76%; p = 0.4). The 15-year freedom from cardiac reoperation was 59%, and 68% and 75% for mitral and aortic reoperation, respectively. Significant risk factors for reoperation were the use of a bioprosthetic valve (p = 0.003) and female gender (p = 0.03). Freedom rates from endocarditis, thromboembolic and bleeding complications at 15 years were 90%, 92%, and 96%, respectively. Among survivors, 95% were in NYHA class I/II. CONCLUSION: Children with rheumatic fever and endocarditis may require simultaneous AVR and MVR. Although the operative mortality is acceptable, patients continue to have constant attrition with time, especially those who have received mechanical prostheses. The risk of cardiac reoperation requirement is high in all patients. Despite the greater need for reoperation, bioprosthetic valves could be offered to selected patients, such as females and those who are non-compliant with anticoagulation regimens.

Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries.

BACKGROUND: In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. HYPOTHESIS: GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). METHODS: We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). RESULTS: Forty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=-18.4±2.5% vs. -20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=-16.9±4.8% vs. -20.4±7.0%, p=0.013) were significantly reduced. CONCLUSIONS: After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality.

Tetralogy of Fallot, Down's Syndrome, Left Ventricular Noncompaction, and Multiples Thrombi.

We report a unique case of left ventricular noncompaction (LVNC), tetralogy of Fallot, and multiples biventricular thrombi in a Down's syndrome. Of interest, speckle tracking analysis detected an abnormal LV myocardial longitudinal deformation, despite the normal ejection fraction and absence of NC at the baseline evaluation.

Obstruction of a percutaneous pulmonary valve by an Aspergillus mycotic thrombus mimicking massive pulmonary embolus.

New technologies in the management of congenital and acquired heart disease may be associated with unfamiliar complications that may require repeated surgical intervention. A high index of suspicion for unusual problems and close collaboration between cardiologists and surgeons is essential when adopting evolving technologies. We report the case of near total obstruction of a 2.5-month-old implanted Melody percutaneous pulmonary valve (PPV) with a thrombus mimicking saddle embolus causing right heart failure and hemodynamic collapse. This obstruction necessitated emergency surgery and homograft replacement. Cultures and pathologic examination revealed fungal thrombus with Aspergillus fumigatus. Subsequently, the patient had rapid improvement, received antifungal treatment, and has shown excellent cardiac and systemic recovery up to 1 year after surgery.

Estimation of modified concordance ratio in sib-pairs: effect of consanguinity on the risk of congenital heart diseases.

Family studies are widely used for research into genetic and environmental influences on human traits. In this paper, we establish statistical methodology for the estimation of a new measure of sib similarity with respect to dichotomous traits measured on each member of within family sib-pair. We call this parameter "excess risk." For inference problems involving a single sample, we construct a large sample confidence interval on the concerned parameter. It has long been suspected that consanguinity is a risk factor for many genetic defects. Therefore, we establish a procedure to test the significance of the difference between excess risk parameters in a sample of consanguineous marriages and another sample of non-consanguineous marriages. We apply the methodology to data from a hospital-based congenital heart defects registry in Saudi Arabia, a population in which consanguinity is quite common.

Aortic and mitral valve replacement in children: is there any role for biologic and bioprosthetic substitutes?

OBJECTIVE: The ideal valve substitute in children does not exist. Biologic and bioprosthetic valves do not require anticoagulation, however their use is complicated by accelerated degeneration and requirement for reoperation. We examine results following mitral (MVR) or aortic (AVR) replacement with biologic and bioprosthetic valves at our institution. METHODS: Medical records of children who underwent AVR or MVR from 1986 to 2006 were reviewed. Median follow-up duration was 10.5 years. Competing-risks methodology determined time-related prevalence and associated factors for three mutually exclusive end states: death, valve reoperation, and survival without subsequent reoperation. RESULTS: One hundred and ten children (age 15.6+/-2.6 years, 80% females) underwent 123 valve replacements with biologic and bioprosthetic substitutes including 87 MVR and 36 AVR (13 had both). Underlying pathology was mainly rheumatic fever (91%). Thirty-nine patients (35%) had undergone a previous cardiac surgery. Most common mitral substitute was Hancock (73%) and homograft (8%); most common aortic substitute was homograft (41%) and Carpentier-Edwards (39%). Competing-risks analysis showed that 15 years after valve replacement, 16% of patients had died without subsequent reoperation, 66% underwent valve reoperations, and only 18% remained alive without further reoperation. Factors associated with increased reoperation risk included younger age at surgery (p=0.005), AVR (p=0.005), male gender (p=0.02) and homograft use (p=0.007) especially in the mitral position (p=0.002). Fifteen-year freedom from endocarditis was 97% while freedom from bleeding and thrombo-embolic complications was 100%. Majority of patients (95%) were in NYHA functional classes I/II at last follow-up. CONCLUSION: While valve reoperation is inevitable following AVR and MVR with biologic and bioprosthetic substitutes; favorable results such as low valve-related morbidity rate, good long-term survival and functional status encourage their consideration as valid replacement alternatives in selected children especially females. Valve durability is higher in the mitral position and longevity of bioprosthetic valves is greater than that of homografts especially in the mitral position.

Surgical strategy to establish a dual-coronary system for the management of anomalous left coronary artery origin from the pulmonary artery.

BACKGROUND: Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation. METHODS: Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed. RESULTS: Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p < 0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p < 0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p < 0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p < 0.00001). CONCLUSIONS: Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.