Anomalous left coronary artery arising from the pulmonary artery discovered beyond infancy.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a congenital heart defect rarely diagnosed beyond infancy. We present a 9-year-old girl who had once been diagnosed as congenital coronary artery fistula. Echogenic mitral chordae tendineae, multiple coronary collaterals within the ventricular septum and free ventricular wall, and a shunting flow from the left coronary artery into the pulmonary artery were prominent echocardiographic features of ALCAPA. Reimplantation of the left coronary artery directly onto the aorta achieved a favorable outcome with regression of left ventricular size and alleviation of mitral regurgitation.

A rabbit model to study regression of ventricular hypertrophy.

BACKGROUND: The purpose of this study is to develop and characterise a reproducible rabbit model of LVH regression following pressure-induced myocardial hypertrophy. MATERIALS AND METHODS: Without endotracheal intubation and mechanical ventilation, a median sternotomy was performed. The median incision was made exactly along the midline of the sternum. Ascending aortic diameter was reduced 50% above the aortic valve, which lead to an approximate 75% reduction in crosssectional area. The sham-operated rabbits underwent the same procedures without actual ligation of the aorta. The animals of early DB group and late DB group were subjected to the second operation for removing the ligation eight weeks or 16 weeks after the initial banding surgery, respectively. Echocardiography, haemodynamic assessment, histology, and electron microscopy were used to assess functional and structural aspects of myocardial hypertrophy at each time point. RESULTS: The pressure overload resulted in robust LVH assessed by echocardiography, haemodynamic assessment, LV weight/body weight ratios, histology, and electron microscopy. Ascending aortic debanding completely or partially reversed LVH. The degree of LVH regression was association with the duration of pressure overload, proved by the fact that restoration of LV structure and function was complete in animals subjected to eight weeks of aortic banding but incomplete in animals subjected to 16 weeks of aortic banding. The animals did not experience severe mechanical ventilatory impairment. CONCLUSIONS: These results demonstrate an efficient and reproducible method of promoting LVH regression in rabbits without endotracheal intubation and mechanical ventilation.

[Association between nuclear factor of activated T cells 1 gene mutation and simple congenital heart disease in children].

OBJECTIVE: To elucidate association between the mutation of nuclear factor of activated T cells 1 (NFATC1) gene in IPT-NFAT region and simple congenital heart disease (CHD) in children. METHOD: We used polymerase chain reaction (PCR) and the sequencing reaction to detect the mutations on the patients and their parents and (or) siblings. RESULTS: PCR amplification of the exon 7 region showed that 2 bands are obtained in 58% of patients with CHD and in 74% of their healthy parents and (or) siblings. Sequencing of the 2 bands revealed that both are amplicons of the exon 7 region, and that the additional band harbors an additional 44 nucleotides segment in the intronic region. The homozygous form of this allele was only present in patients with ventricular septal defect (2/24), atrial septal defect (3/18) and bicuspid aortic valve (1/4) in which G to A transition at nucleotide 17 of the third 44 bps was found. Neither the unrelated non-CHD individuals nor the ones with other CHD showed positive presence for the homozygous form of this allele. CONCLUSIONS: There is a differential amplification of a tandem repeat region in intron 7 of NFATC1 and homozygous form of this allele in patients with ventricular septal defect, atrial septal defect and bicuspid aortic valve. NFATC1 gene may be an a susceptibility marker for ventricular septal defect, atrial septal defect and bicuspid aortic valve.

[The role of European system for cardiac operative risk evaluation in the prediction of quality of life in patients after coronary artery bypass graft surgery].

OBJECTIVE: To assess the value of European system for cardiac operative risk evaluation (EuroSCORE) in predicting quality of life in patients post coronary artery bypass graft surgery (CABG). METHODS: A total of 387 patients underwent CABG in our institute from December of 2002 to December of 2007 were assessed by EuroSCORE before operation. Health-related quality of life (QoL) was estimated postoperatively with Seattle angina questionnaire (SAQ), Nottingham healthy profile (NHP) and Duke activity status index (DASI) in order to evaluate the value of EuroSCORE for predicting quality of life in patients post CABG. RESULTS: There were statistically significant but weak correlations between postoperative QoL score and preoperative EuroSCORE score (r: 0.010 - 0.276). Emotional and psychological experience subgroup analysis showed better predictive value of EuroSCORE score on postoperative QoL score in improved physical functioning subgroups (r > 0.2). Linear regression analysis showed that EuroSCORE score was significant but weakly (r(2) < 0.1) correlated with postoperative QoL score (P < 0.05). CONCLUSION: Preoperative EuroSCORE score is weakly correlated with postoperative QoL score in patients post CABG.

A novel mutation in GATA4 gene associated with dominant inherited familial atrial septal defect.

OBJECTIVE: Atrial septal defect (ASD) is a common congenital heart disease (CHD). Although most cases are sporadic, familial cases have been reported. The transcription factors NKX2.5 and GATA4 play important roles in the pathogenesis of ASD. Mutations in either gene have been identified in familial cases of ASD. Here, we examine a Chinese family with isolated ASD to find out whether there is any mutation in NKX2.5 or GATA4 accounting for the etiology. METHODS: We identified kindred spanning 3 generations in which 8 of 31 (38%) individuals had ASD. One hundred seventy unrelated individuals were included as controls. Peripheral blood samples were collected and genomic DNA was extracted from the leukocytes. NKX2.5 and GATA4 were amplified by polymerase chain reaction (PCR) with specific primers. The sequences of PCR products were compared between affected members and unaffected members, as well as controls. RESULTS: Direct sequencing of NKX2.5 from the genomic DNA of family members failed to identify mutations, whereas sequencing of GATA4 identified an A-to-G transition at nucleotide 928 in exon 5 that predicted a methionine to valine substitution at codon 310 (M310V) in the NLS region. All affected members and a patriarch of the family who was recognized as a carrier exhibited this mutation, whereas the other unaffected family members or control individuals did not. This mutation has not been reported previously in either familial or sporadic cases of CHD. CONCLUSIONS: We identified a novel M310V mutation in GATA4 gene that is located in the NLS region and leads to hereditary ASD in a Chinese family. In this family, we identified a carrier with incomplete penetrance and 8 patients with variable expressivity. However, the mechanism by which this mutation contributes to the development of a congenital heart defect remains to be ascertained.

[Influence of pravastatin on blood lipids and serum high sensitive C-reactive protein in patients undergoing conventional coronary artery bypass grafting under on-pump: a clinical study of 81 cases].

OBJECTIVE: To investigate the effect of pravastatin on blood lipids and serum high sensitive C-reactive protein (HsCRP) in patients undergoing conventional coronary artery bypass grafting under on-pump bypass (CCABG). METHODS: Eighty-one patients underwent CCABG. Among which 40 took orally pravastatin 20 mg once daily to at least 28 days after operation, and 41 were used as control group. The serum levels of total cholesterol (TC), triglyceride (TG), HDL-C cholesterol (HDL-C), LDL-C cholesterol (LDL-C), and HsCRP were monitored before and 24 h, 72 h, 7 days, 10 days, 14 days, and 28 days postoperatively. RESULTS: In the control group the levels of different blood lipids after operation remarkably decreased after operation compared with those before operation (all P < 0.05), reached the lowest levels 24 h after operation, then gradually increased, however, still lower than those before operation (all P < 0.05), and recovered to the baseline level 28 hours after operation; and the HsCRP level increased 24 h after operation and peaked 72 h after, then gradually decreased, and recovered to the baseline level 28 days after operation. In the pravastatin group the TC level reached its lowest level 24 h after operation, then gradually increased, however, still lower than that before operation, and recovered to the baseline level 28 days after operation; and the TG level reached the lowest level 24 h after operation (P < 0.05), and then gradually increased 3 d after operation (P > 0.05). The TC, TG, and LDL-C levels 7, 10, 14, and 28 d after operation of the pravastatin group were all significantly lower than those of the control group (all P < 0.05). The HsCRP levels at different time points of the pravastatin group were all significantly lower than those of the control group (all P < 0.05). CONCLUSION: The use of pravastatin in the early stage of CCABG is safe and can decrease systemic inflammatory reaction.

[Change of N-terminal pro-brain natriuretic peptide and big endothelin in patients undergoing coronary artery bypass grafting].

OBJECTIVE: To examine the change in N-terminal pro-brain natriuretic peptide (Nt-proBNP) and big endothelin (big ET) in patients undergoing coronary artery bypass grafting (CABG), and to evaluate their value in predicting postoperative mortality and complication. METHODS: Forty-seven patients undergoing coronary artery bypass grafting under on-pump (CCABG) and 43 patients undergoing off-pump bypass (OPCAB) were included for study. The levels of Nt-proBNP and big ET were determined before and 24 hours after operation in all patients. RESULTS: (1)There were no differences between two groups. The serum levels of Nt-proBNP and big ET increased significantly 24 hours after operation. Compared with those before operation, Nt-proBNP [(1 083.5 +/- 717.9) pmol/L] in CCABG group was increased [(1 579.2 +/- 719.7)pmol/L, t = -4.30, P<0.01], big ET was increased from (1.10 +/- 1.82 ) pmol/L to (1.68 +/- 1.73)pmol/L(t = -5.35, P<0.01) 24 hours after operation; Nt-proBNP [(999.6 +/- 843.6) pmol/L] in OPCAB group was increased [(1 460.8+/-830.0) pmol/L, t = -4.20, P<0.01], big ET was increased from (1.35 +/- 1.65) pmol/L to (1.73 +/- 1.50) pmol/L (t = -2.46, P=0.018) 24 hours after operation. (2)The level of Nt-proBNP before operation was showed to be negatively correlated with left ventricular ejection fraction (LVEF) (r = -0.43, P<0.001). (3)By univariate and multivariate Logistic regression analysis, the association of clinical variable with postoperative complication was assessed. Multivariable predictors, including the level of LVEF (OR = 1.045, 95%CI:0.999-1.092, P = 0.050) and Nt-proBNP 24 hours after operation (OR = 0.990, 95%CI:0.999-1.000, P = 0.014), were significantly associated with a higher postoperative mortality, lower cardiac output, and higher incidence of myocardial infarction and congestive heart failure. Receiver operating characteristic curves (ROC) for Nt-proBNP 24 hours after operation was valid for the prediction of postoperative complication, and the area under the curve was 0.698 (95% CI:0.585-0.811, P<0.003), sensitivity and specificity were 88.9% and 57.1%, respectively. CONCLUSION: Significant increase in Nt-proBNP and ET is found after CABG. BNP and LVEF are showed to be risk factors for postoperative complications in patients undergoing CABG.

[Comparative studies of the blood lipids and apolipoproteins A1 and B in patients underwent coronary artery bypass grafting under on-pump and off-pump bypass].

OBJECTIVE: To compare the influences of coronary artery bypass grafting under on-pump (CCABG) and off-pump bypass (OPCAB) on blood lipids and Apolipoproteins A(1) and B. METHODS: 41 patients underwent CCABG and 38 patients OPCAB. The serum levels of total cholesterol (TC), triglycerides (TG), HDL-C cholesterol (HDL-C), LDL-C cholesterol (LDL-C) and Apolipoproteins A(1) and B were monitored before and 24 h, 72 h, 7 d, 10 d, 14 d, 28 d after operation in all patients. None of them received lipid-lowering drug during the test. RESULTS: (1) There were no difference between two groups. The serum levels of TC, LDL-C decreased significantly in 24 h, 72 h, 7d, 10 d, 14 d after operation (P < 0.001). The level in 24 h was the lowest, then began increasing; until 28 days after operation the serum levels of TC, LDL-C exceeded preoperative baseline level (P < 0.05). (2) the level of TG and HDL-C in 24 h was the lowest (P < 0.001), then began increasing (P > 0.05). Until 28 days after operation the serum levels of TG exceeded preoperative baseline level (P < 0.05). (3) the serum levels of ApoA(1) decreased significantly in 24 h, 72 h, 7 day, 10 day, 14 day after operation (P < 0.001). The level in 24 h was the lowest, and lasted until 28 days after operation (P > 0.05); the serum levels of ApoB in 24 h was the lowest, then began increasing (P > 0.05). CONCLUSIONS: There presents significant influence on blood lipids and Apolipoproteins A(1) and B after CABG, and initiate lipid (s)-lowering therapy as soon as possible.

[Changes of interleukin-1beta and tumor necrosis factor-alpha of right atrial appendages in patients with rheumatic valvular disease complicated with chronic atrial fibrillation].

OBJECTIVE: To investigate whether atrial expression of interleukin-1beta (IL-1beta) and tumor necrosis factor-alpha (TNF-alpha) of right atrial appendages are altered in patients with rheumatic valvular disease during chronic atrial fibrillation. METHODS: A total of 48 patients with rheumatic heart disease were included. 27 patients had no history of atrial fibrillation, 21 patients had atrial fibrillation. Atrial tissue was obtained from the right atrial appendage during open heart surgery. The protein expression of IL-1beta and TNF-alpha was detected by immunohistochemistry method. The fibrosis of right atrial appendage was detected by Masson staining. RESULTS: The fibrosis of right atrial appendage was significantly increased in patients with chronic atrial fibrillation. The protein expression of IL-1beta and TNF-alpha were significantly increased in patients with chronic atrial fibrillation. CONCLUSIONS: The protein expression of IL-1beta and TNF-alpha were significantly increased in patients with rheumatic valvular disease during chronic atrial fibrillation. Inflammation may be one of the mechanisms for the development and persistence of atrial fibrillation.