Case series: Criss-cross Heart

Criss-cross heart (CCH) is a rare cardiac malformation which is characterized by crossing of the inflow streams of the two ventricles due to rotation of ventricular axis. The anomalies can be identified both atrioventricular concordance and discordance. The etiology of CCH is remaining unknown. Prominent symptom is cyanosis. The primary investigation is transthoracic echocardiography to identify this abnormality. Many patients need further investigation to review anatomy. Total correction is the aim for treatment but it is difficult for this complex anatomy. There were 5 patients who visited cardiology clinic at Queen Sirikit National Institute of Child Health (QSNICH) from 2002 to 2017. The objective is to review the treatment options that we performed in CCH. Most common associated anomalies were double outlet right ventricle and pulmonary stenosis. One patient died before surgical intervention; others were performed palliative surgery.

Kết quả dài hạn sau phẫu thuật tứ chứng fallot tại Trung tâm Tim mạch - Bệnh viện Nhi Trung ương

Đặt vấn đề: Phẫu thuật sửa toàn bộ tứ chứng Fallot đã được tiến hành tại Việt Nam với kết quả ngắn hạn khả quan, tuy nhiên kết quả lâu dài sau phẫu thuật thì vẫn chưa được làm rõ. Nghiên cứu này của chúng tôi được tiến hành nhằm đánh giá kết quả dài hạn sau phẫu thuật sửa toàn bộ tứ chứng Fallot tại Trung tâm Tim mạch - Bệnh viện Nhi Trung ương. Đối tượng - phương pháp: Các bệnh nhân được phẫu thuật sửa toàn bộ tại Trung tâm Tim mạch - Bệnh viện Nhi Trung ương trong thời gian từ tháng 12 năm 2006 đến tháng 12 năm 2020 được thu thập bệnh án và được đưa vào nghiên cứu. Kết quả: Trong thời gian nghiên cứu, có tổng số 532 trường hợp được phẫu thuật sửa toàn bộ tứ chứng Fallot tại Trung tâm Tim mạch - Bệnh viện Nhi Trung ương. Tuổi phẫu thuật trung bình của các bệnh nhân trong nhóm nghiên cứu là 11.7 tháng (8.8 - 17.4). Trong nhóm nghiên cứu có 11 trường hợp tử vong sớm sau phẫu thuật (2.1%) và 2 trường hợp tử vong muộn sau phẫu thuật (0.4%). Tỷ lệ bệnh nhân cần mổ lại trong nhóm nghiên cứu là 2.3% (12 bệnh nhân). Thời gian theo dõi trung bình sau phẫu thuật là 40.4 ± 26.27 tháng. Triệu chứng suy tim trên lâm sàng theo Ross tại thời điểm khám lại cuối cùng cho thấy phần lớn các bệnh nhân không có biểu hiện suy tim trên lâm sàng, chỉ có duy nhất 1 bệnh nhân Ross - III. Kết quả siêu âm kiểm tra tại thời điểm khám lại cuối cùng cho thấy có: 144 bệnh nhân (27%) có hở van ĐMP tự do, 42 bệnh nhân (7.9%) hở phổi mức độ trung bình, có 90 bệnh nhân (16.9%) có tình trạng giãn thất phải sau mổ ở các mức độ, và 2 trường hợp hở van ba lá mức độ trung bình nặng. Kết quả kiểm tra điện tim cho thấy hình ảnh block nhánh phải chiếm tỷ lệ 54.2%; hình ảnh giãn thất phải chiếm tỷ lệ 68.2%, và 2.3% bệnh nhân có hình ảnh block nhĩ thất độ I. Kết luận: Kết quả lâu dài sau phẫu thuật sửa toàn bộ tứ chứng Fallot tại Trung tâm Tim mạch - Bệnh viện Nhi Trung ương là tốt. Theo dõi lâu dài sau phẫu thuật là cần thiết đối với nhóm bệnh tim bẩm sinh phức tạp này nhằm đánh giá nguy cơ tử vong muộn và các yếu tố liên quan tới mổ lại của bệnh nhân. ABSTRACT RESULTS OF TOTAL CORRECTION FOR TETRALOGY OF FALLOT IN CHILDREN HEART CENTER - NATIONAL CHILDREN’S HOSPITAL VIETNAM Objective: Long term results of treatment for total correction of tetralogy of Fallot is unknow in Vietnam. We conduct this study to evaluate the long - term results of surgical treatment for tetralogy of Fallot in Children Heart Center, National Children Hospital, Hanoi, Vietnam. Methods: A retrospective study was conducted to evaluate the long - term outcome after total correction for tetralogy of Fallot in Children Heart Center - National Children Hospital, Hanoi, Vietnam since December 2006 to December 2020. Results: There were 532 patients operated during the study period. The median patient age was 11.7months (8.8 - 17.4). The hospital mortality was 11 patients (2.1%), and the late mortality was 2 patients (0.4%). There were 12 patients required re - operation during follow up, with the median time of follow - up was 40.4 ± 26.27months. According to Ross classification about heart failure, the majority of the patient have no heart failure, and only 1 patient have Ross - III. The echocardiography at the last visit shows: 144 patients (27%) have free pulmonary regurgitation, 42 patients (7.9%) have moderate pulmonary regurgitation, and 90 patients (16.9%) have right ventricle enlargement, with 2 patients have severe tricuspid valve regurgitation. The electrocardiogram shows: right bundle block was 54.2%; right ventricular dilation was 68.2%, and 2.3% of the patient have first degree of atrioventricular block. Conclusion: Long term results of total correction for tetralogy of Fallot in Children Heart Center, National Children Hospital are good. Further investigation is needed to evaluate the late mortality and morbidity in this complex heart disease. Key words: Tetralogy of Fallot, total correction, long term results.

Mortality Risk Factors in Tetralogy of Fallot Patients Undergoing Total Correction

A total correction is a preferred treatment for Tetralogy of Fallot patients in every part of the world. However, the mortality in developing countries was as high as 6.9% to 15.3%. This was a retrospective analytic study that analyzed pre and post-operative risk factors that affected mortality on TOF patients that were performed total correction in Indonesia. A total of 47 TOF patients that were performed total correction from January 2016 to September 2019 were enrolled in this study based on the inclusion criteria. Preoperative and post-operative data were obtained from medical records. In this research, the majority of mortality was found in male patients (39.3%), while the female’s rate was lower (36.8%). Overall mortality was 38.3% and one operative death was found. The average age of patients was 84.12 months (12-210 months), whereas the average height (85.56 ± 36.17cm vs. 112.93 ± 21.73) and weight (17.22kg vs. 28.21kg) were lower for mortality patients. Some significant preoperative variables were identified as mortality risk factors such as: age below 60 months (p=0.047), smaller weight and height (p=0.008; p=0.002), abnormal hematocrit (p=0.002), and oxygen saturation below 75% (p=0.018). Significant post-operative risk factors included: temperature above 38.5⁰C (p=0.000), and ventilator time of more than 48 hours (p=0.033). In conclusion, the mortality of TOF patients undergoing a total correction in developing countries was quite high. It was associated with some risk factors, such as younger age, lower weight and height, low oxygen saturation, post-operative fever, and prolonged ventilator time.

The effect of pupil diameter on the axial length of the eye in children with myopia using orthokeratology lenses

Background. In recent years the refractive therapy with orthokeratology lenses (OKL) has become the most popular among the effective ones. The efficiency of the method is determined by several initial parameters of a patient, including the diameter of the pupil. The purpose was to study the influence of pupil diameter on the eye axial length in patients with myopia who use orthokerato­logy lenses. Materials and methods. The examination involved 120 children divided into 2 groups. The group I used OKL, group II used glasses with total correction. Results. Group I demonstrated a strong correlation between the diameter of the pupil and myopia progression gradient; group II showed no correlation. Conclusions. The most effective method of control for an advanced form of myopia and baseline dimension of the lens less than 4.52 mm is an application of refractive therapy with orthokeratology lenses.

TRANSCATHETER VENUS P VALVE IMPLANTATION AT PULMONARY POSITIONPOST TOF REPAIR WITH SEVERE PR- INITIAL CASE IN PAKISTAN

After total correction for tetrolgy of fallot (TOF), right ventricle behaves in an unpredictable manner depending on type of right ventricular outflow tract (RVOT) reconstruction and surgical expertise of infundibular muscle resection. We are reporting a 23 years old girl who underwent total correction at two years of age. RVOT was reconstructed with native pericardial patch. Gradually she developed breathlessness and occasional chest pain. Echocardiograghy revealed hugely dilated right ventricle (RV) with gross pulmonary regurgitation and RV dysfunction. Cardiac MRI also calculated right ventricular end systolic volume (RVESV) 57 ml/m2 and right ventricular end diatolic volume (RVEDV) 157ml/m2.We decided to implant transcatheter venus p-valve at pulmonary position. The procedure went successful having competent pulmonary valve and improved RV function. Total fluoro time was 36.4 minutes and total procedural time was two hours. This procedure was done first time in Pakistan with optimal results.

Left ventricular rotational abnormalities in adult patients with corrected tetralogy of Fallot following different surgical procedures – a three-dimensional speckle-tracking echocardiographic study

Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Abnormal aortic dimensions and elasticity parameters have been long described for corrected TOF (cTOF) together with left ventricular (LV) rotational abnormalities. The present study focuses on investigating LV rotational mechanics in cTOF, and possible correlation of these parameters with aortic elastic properties. It was also aimed to be examined whether different surgical strategies has any effect on the results. Methods The study involved 26 adult cTOF patients, from which 14 had palliative surgery first [Blaloc-Taussig (n=10), Waterstone-Cooley (n=2) shunts, or Brock procedure (n=2)] (mean age at the repair: 7.3±10.2 years) and a late total correction (mean age at the repair: 10.0±13.3 years) (pcTOF), while the early total correction was the treatment of choice in 12 patients (mean age at the repair: 4.2±3.2 years) (etrTOF). Their results were compared to that of 37 age- and gender-matched healthy adults. Routine transthoracic two-dimensional Doppler echocardiography extended with assessment of aortic elastic properties and three-dimensional speckle-tracking echocardiography (3DSTE) was performed in all cTOF patients and controls. Results Sixteen out of 26 cTOF patients showed normally directed LV rotational mechanics, while apical and basal LV rotations were in the same clockwise and counterclockwise directions in 7 and 3 cTOF cases, respectively (38%). This sort of LV movement is called as LV rigid body rotation (RBR). The ratio of LV-RBR between etrTOF and pcTOF patients did not differ significantly (33% vs. 43%, p=0.70). Significantly reduced LV apical rotation (5.2±3.7 degree vs. 10.2±4.5 degree, p<0.05) and twist (9.0±3.3 degree vs. 14.6±4.9 degree, p<0.05) could be demonstrated in cTOF patients with normally directed LV rotational mechanics with preserved LV basal rotation regardless of previous procedure. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases (3.2±3.7 degree vs. 7.1±2.5 degree, p<0.05). From the 7 cTOF patients with clockwise LV-RBR, apical and basal LV rotation proved to be −6.8±5.4 degree and −4.9±2.4 degree, respectively with LV apico-basal gradient of 4.3±3.6 degree. The mean LV apical and basal LV rotation of 3 cTOF patients with counterclockwise LV-RBR prove to be −2.1±3.4 degree and 0.2±0.9 degree, respectively with LV apico-basal gradient of 2.5±2.4 degree. Significant correlations could be demonstrated between LV apical rotation and aortic stiffness index (r=−0.55, p=0.03) and aortic distensibility (r=0.52, p=0.04). Conclusions Significant LV rotational abnormalities could be demonstrated in cTOF with the high prevalence of LV-RBR. cTOF patients with early total reconstruction proved to have beneficial results. Abnormal physiologic response of LV rotational mechanics to increased aortic stiffness can be detected in cTOF patients without LV-RBR. Funding Acknowledgement Type of funding source: None