For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.
It has been shown that both rest and stress myocardial perfusion imagings with technetium agents can be performed on the same day using two different doses injected within few hours. The purpose of this study was to compare the two protocols (stress-rest and rest-stress) in detecting coronary artery diseases. One hundred and sixty patients (101 males, 59 females, mean age $57{\pm}9$ years) and 120 patients (79 males, 41 females, mean age $59{\pm}10$ years) underwent stress-rest myocardial perfusion SPECT and rest-stress myocardial perfusion SPECT, respectively. All of them underwent both myocardial perfusion SPECT and coronary angiography within 1 month. A coronary stenosis was considered significant when it compromised the luminal diameter by ${\geq} 50%$. The chi square test was used to compare differences in sensitivity, specificity and accuracy between the two groups. The overall sensitivity, specificity and accuracy of stress-rest protocol were 99%, 35% and 68%, respectively. Those of rest-stress protocol were 96%, 47% and 78%, respectively. There was no difference between the two protocols in identifying individual diseased coronary artery branches. Therefore, one day stress-rest and rest-stress myocardial SPECT using $^{99m}Tc$ agents were comparable and were very sensitive tests in detecting coronary artery diseases.
Gated SPECT can evaluate the regional wall motion of the heart. We evaluated the regional wall motion of the perfusion abnormality in conventional perfusion SPECT with gated SPECT. In case of suspicious perfusion abnormalities, we tried to differentiate the artifact from true abnormality in coronary vascular disease using gated SPECT. We thought that artifacts would have normal wall motion, whereas fixed defects with decreased wall motion would probably represent coronary artery disease. A total of 275 patients who were performed coronary angiography and T1-201 rest/Tc-99m MIBI dipyridamole stress gated SPECT within 2 months were enrolled. In coronary angiography, stenosis more than 50% was considered as coronary artery disease. After injection of 111MBq T1-201 rest image was obtained on triple head SPECT system. 370MBg Tc-99m MIBI was used for the stress image. Eight-frame per-cardiac-cycle gated Tc-99m SPECT studies were done. All the images were analyzed visually. Using perfusion SPECT, the overall sensitivity and specificity were 87% and 55% respectively. Regarding artery territory, sensitivity and specificity were 68% and 73% for left anterior descending artery(LAD), 62% and 78% for right coronary artery(RCA), 42% and 90% for left ciramflex artery(LCX). Using gated SPECT, the overall sensitivity and specificity were 87% and 66% respectively. Sensitivity and specificity were 68% and 78% for LAD, 62% and 79% for RCA, 42% and 90% for LCX. Among 21 false positive cases in perfusion SPECT, 5 cases were interpreted as true negative with gated SPECT. We conclude that gated SPECT provides a valuable adjunct to perfusion SPECT in characterizing perfusion abnormalities and to improve specificity.
Ji, Min-Chul;Park, Se-Jin;Choi, Jae-Young;Ko, Young-Guk;Kim, Myoung-Soo;Kim, Ji-Hong;Shin, Jae-Il
Childhood Kidney Diseases
/
v.14
no.2
/
pp.223-229
/
2010
Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.
Between 1974 and 1982, 31 patients from 7 to 15 years of age have undergone valve replacement for their acuqired cardiac valvular diseases at Seoul National University Hospital. Furteen patients (45.2%) had a definite history of rheumatic fever and only 4 patients (12.9%) had atrial fibrillation on their preoperative electrocardiograms. Characteristically, the valvular lesions were ones of insufficiency with or without associated stenosis in all patients except only one whose mitral valve was tightly stenotic. Thrity-seven valves were replaced in 31 patietns including a case of successful replacement of his failed xenogragt mitral valve : 4 mechanical valves were used in 3 patients and 33 xenograft valves were used in the remaining 28 patients. The size of the valves were not the major problem at the time of opertion because most of the patients had a dilated heart from disease. There were 3 diaths within 30 days of surgery (9.7% operative mortality rate) and 3 late deaths (9.7% late mortality rate) with an overall mortality rate of 19.4%. Twenty-eight early survivors were followed up for a total of 488 patient-months. Thromboembolic complications occurred in 5 patients with 2 deaths: cmbolic rate of 17.9% or the actuarial embolic incidence of 12.29%/patients-year. four xenograft tissue valves in 4 patients had failed during the period from 19 to 41 months of surgery with an overall valve failure rate of xenograft of 14.3% or the actuarial incidence of 9.84% failure/patient-year. One of these 4 patients had required replacement of his failed mitral xenograft valve which had severe calcification and tissue disruption with primary tissue failure rate of 3.6% or the actuarial incidence of 3.13% failure/patient-year. The actuarial survial including the operative morality was 50.0% at 5 years of surgery. /the actuarial incidence free from thromboembolism in bioprosthetic group was 85.4% at 42 months, while it was 33.4% in mechanicial group at 60 months after operation. The actuarial incidence free from overall valve failur of 100.0% until 18 months after surgery was followed by a rapid decrease during the next 2-year period, and it was only 17.8% at the follow-up end of 42 months after surgery. It was suggested that the major advantage of low thrombogenecity with xenograft valve should be balanced against the high incidence of accelerated valve failure when it is used in children whose age is younger than 15 years old. The possible role of recurrent rheumatic attacks to the early failure of xenograft tissue valve was also discussed.
A variety of experiments concerning the development of ideal prosthetic grafts for correcting circumferential tracheal defects have been performed. The requirements for an ideal tracheal prosthesis are impermeability to air, consistency to prevent collapse, and acceptance by the host tissue causing a minimum inflammatory reaction, allowing fibroblastic infiltration and epithelialization. The synthetic material, polyurethane(PU), is known as a biocompatible polymer with an inert component. In this study, the tracheal prosthesis was made from microporous PU(30 micrometer in diameter) coated with gelatin and reinforced with isoplastic rings. This procedure provides the prosthesis with a compression strength. The out side diame er of the prosthesis was 20 mm with a length of 30 mm. The gelatin used in the study was obtained from pig skin and immobilized and cross-linked by irradiation(60 Co gamma ray) to promote host tissue incorporation and render the prosthesis epithelization after implantation. Animal experiments using 10 mongrel dogs were performed to compare three kinds of prosthesis; gelatin coated polyurethane graft, uncoated polyurethane graft, and prosthesisf pericadium complex graft. After 6 weeks of implantation, the epithelialization of implants was seen on the gelatin-coated and prosthesisfpericadium complex grafts. Implanted prosthesis were complicated by airway obstruction due to anastomosis granuloma. Early tracheal stenosis was found in the uncoated graft group. Two kind of anastomosis techniques were tested on the gelatin-coated prosthesis. Everted anastomosis resulted severe granuloma than the inverted anastomosis. In the prosthesislpericadium complex graft, bacteria and inflammation at a anastomotic site was found. Based on these results, gelatin coated porous polyurethane trachea prosthesis is biocompatible and may be useful in clinical application with further investigation.
Obstructive intracardiac lesions, like mitral stenosis or insufficiency (MR), by myxomas of the left atrium have been commonly reported, but the attenuation of MR by myxoma combined with coronary artery disease is very rare. We report a 70-year-old female patient whose left atrial myxoma had attenuated moderate MR to mild MR and required mitral valve surgery after removal of the myxoma. She also had coronary artery disease, severe pulmonary hypertension and moderate tricuspid regurgitation due to the mitral valve lesions obstructed by myxoma. The patient underwent removal of myxoma, mitral and tricuspid valve reconstructions, and coronary artery bypass grafting. She was discharged at the postoperative 14 day without any problems.
Purpose: $^{99m}Tc$-tetrofosmin(TF) is a recently proposed myocardial imaging agent which has similar biokinetic characteristics to $^{99m}Tc$-sestamibi(MIBI). The aim of this study was to compare TF and MIBI myocardial perfusion SPECTs in detecting coronary artery disease. Materials and Methods: One hundred and sixty patients(101 males, 59 females, mean age $57{\pm}9yr$) who had undergone both myocardial perfusion SPECT(M-SPECT) and coronary angiography within 1 month were studied. M-SPECT was performed using TF in 115 patients and MIBI in 45 patients. Stress-rest one day protocol was used in all patients. A coronary stenosis was considered significant when the luminal diameter ${\geq}$50% was compromised. The chi square test was used to compare differences in sensitivity and specificity between the two groups. Results: There was no difference in age and diseased coronary artery branches between the two groups. There were more male patients in TF group: Male-to-female ratios of TF and MIBI groups were 78:37 and 23:22, respectively(p<0.05). The overall sensitivity of TF study was 92% and specificity 36%. The overall sensitivity and specificity of MIBI study were 93% and 30%, respectively. There was no difference between TF and MIBI groups in identifying individual diseased coronary artery branches. Conclusion: One day stress-rest myocardial SPECT using either TF or MIBI was comparable and was a very sensitive test in detecting coronary artery disease.
Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with $^{99m}Tc$-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade I to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal lobes being the most frequently involved site. $4{\times}4$ pixel sized ROIs were applied on the frontotemporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as: PI (%)=average F-T activity/average cerebellar activity${\times}100$ Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean: $74.3{\pm}17%$) and increased significantly after operation ($81.4{\pm}17%$, p<0.001). Individual post-EDAS PI improved in 15/21 cases, showed no significant change in 5 and was slightly aggravated in 1. The amount of clinical improvement (${\Delta}CI$) was graded with a scale of 0 to 4 based on frequency and severity of TIA attacks. When patients were grouped according to pre-EDAS PI, group II (PI 70-89) showed a significantly higher ${\Delta}CI$ (3.3) compared to group I (PI< 70, 1.57) or group III (PI >90, 0.5) (P< 0.001). The amount of perfusion improvement (${\Delta}PI$) showed significant correlation with ${\Delta}CI$ (r=0.42, p=0.04). ${\Delta}PI$ did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.
A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The post-operative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.
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