SURGICAL MANAGEMENT OF A NEONATE WITH INTERRUPTED AORTIC ARCH, TRANSPOSITION OF THE GREAT ARTERIES, AND TRICUSPID ATRESIA

A critically ill infant presented to our Center with congestive heart failure due to Type A interrupted aortic arch, D-transposition of the great arteries, tricuspid atresia, a large ventricular septal defect, and a closing ductus arteriosus. Partially corrective surgery including aortic arch reconstruction, ductal division, and pulmonary artery banding was successful. Future total correction is planned.     

Sudden unexpected death in children with congenital heart disease.

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.     

犬心右室,左室大面积梗塞时的容量负荷评价

在１２只犬,结扎四支冠脉,造成犬心右室、左室大面积梗塞和心源性休克时,左室收缩压（ＬＶＳＰ）及最大正负压力阶差（±ｄｐ／ｄｔｍａｘ．）分别下降５４％、５１％和４７％,而右室收缩压（ＲＶＳＰ）及±ｄｐ／ｄｔｍａｘ．仅降低９％、２５％和２７％。组Ⅰ（６只犬）快速扩容（低分子右旋糖酐３０ｍｌ／ｋｇ,２０ｍｉｎ内静脉输入）,结果右室反向搏动增强,双心室±ｄｐ／ｄｔｍａｘ．进一步降低,右房压（ＲＡＰ）及左室舒张末压（ＬＶＥＤＰ）极度升高达２．９±０．２ｋＰａ和５．０±０．３ｋＰａ（Ｐ均＜０．０１）,甚至诱发室颤。组Ⅱ缓慢静点多巴胺（１０μｇ／ｋｇ·ｍｉｎ）和硝酸甘油（１μｇ／ｋｇ·ｍｉｎ）３０ｍｉｎ,有效提高了动脉压（ＡＰ）,心输出量（ＣＯ）,ＬＶＳＰ及左室±ｄｐ／ｄｔｍａｘ．使休克逆转。结果表明,大面积左、右室梗塞伴休克时,右室残余心肌的代偿性收缩仍能造成ＲＶＳＰ与右室泵功能呈分离状态;此时快速扩容将进一步损害左、右室功能,而联合使用硝酸甘油和多巴胺能有效纠正休克同时不造成ＲＡＰ和ＬＶＥＤＰ的升高。     

Clinico-experimental study of histoenzymological changes in the ventricular myocardium in pulmonary embolism]

The SDH and LDH activity in ventricular myocardium was studied in early autopsies of 16 patients, which died from acute pulmonary thromboembolism, and also in 17 dogs with experimental pulmonary embolism. In general the data of histoenzymological study of experimental and sectional material were identical. We revealed some factors, which correlated with low activity of catabolic enzymes in ventricular myocardium of the patients with pulmonary embolism: small volume of embolic occlusion (the thromboembolism of lobar and segmental pulmonary arteries); the presence of prior chronic cardiopulmonary disease; the advanced age and the female sex. Preexisting cardiopulmonary disease, as well as age and sex changes of myocardial metabolism, may assist the development of heart failure by relatively small volume of embolic obstruction.     

Pulmonary hypertension induced with an intra-arterial balloon: an alternate mechanism

The Laks catheter is a triple-lumen balloon catheter used to distend the canine main pulmonary artery while recording right ventricular pressure and the arterial pressure distal to the balloon. A rise in arterial pressure reported to occur during distension has been attributed to vasoconstriction rather than passive obstruction by the balloon. We tested this in six anesthetized dogs by inflating the Laks catheter-balloon while recording pressure distal to the balloon from the Laks catheter as well as from additional catheters in right and left pulmonary arteries placed retrogradely through lobar branches following thoracotomy. We found that balloon inflation increased pressures in the arterial port of the Laks catheter and in the left pulmonary artery catheter but reduced it in the right pulmonary artery. Tightening a snare around the right pulmonary artery had the same effects on pressures. Similar results were obtained while cardiac output was controlled by left ventricular bypass perfusion in four dogs. We conclude that the Laks catheter-balloon obstructs flow to the right lung and that the arterial pressure rise recorded in it during balloon inflation cannot be distinguished from that caused by occlusion of the right pulmonary artery.     

SURGICAL CORRECTION OF COMBINED DOUBLE-OUTLET RIGHT VENTRICLE AND TAUSSIG-BING SYNDROME

Total surgical correction of a Taussig-Bing type double outlet right ventricle (DORV) was successfully performed in a severely cyanotic 3-year-old girl. The malformation was associated with bilateral conus, d-transposition of the great arteries, d-loop, and a subpulmonary ventricular septal defect (VSD) without significant pulmonary stenosis in situs solitus. It was impossible to create a tunnel repair by resecting the markedly hypertrophied muscular conus that separated the aortic valve from the subpulmonary ventricular septal defect. Therefore, the VSD was repaired with a Dacron patch, transforming the double outlet right ventricle into a transposition, after which total correction was achieved by means of a Mustard procedure.     

The atrioventricular branches of the human coronary arteries

Branches of the coronary arteries of normal human hearts, supplying both atria and ventricles, were found by fine dissection and have been named rami atrioventriculares. They comprise atrial branches from ventricular arteries and ventricular branches from atrial arteries. Their incidence was 74% in the 50 adult individuals studied. The subjects had committed suicide with a poison which did not damage the coronary arteries. The atrioventricular branches constitute communications across the coronary sulcus, thus establishing a continuity between the atrial and the ventricular arterial supply. Therefore, there is not invariably a sharp demarcation of blood supply between atria and ventricles, as has been commonly taught. Neither atrial nor ventricular branches consist exclusively of ascending and descending branches of the coronary arteries, as has been assumed. Atrioventricular branches can play a role in collateral circulation and may, in individuals who are born with them, provide an explanation for some of the variability in signs and symptoms incidental to heart attacks.     

Anatomical correction of complete transposition of the great arteries and ventricular septal defect in infancy.

Two patients, aged 8 weeks and 5 years, with D transposition of great arteries and large ventricular septal defect were treated by transection of both aorta and pulmonary arteries and reattaching them to the appropriate ventricles. This included the origins of the coronary arteries. The ventricular septal defect was closed through a transverse ventriculotomy using a Dacron patch. The younger child was operated on as an emergency because of cyanosis and severe heart failure resistant to intensive medical treatment. The older child had had previous banding of the pulmonary artery at the age of 1 year. In both patients pulmonary artery pressure dropped to below half systemic pressure immediately after the operation. Postoperative progress was satisfactory with relief of cyanosis and heart failure. Early anatomical correction of transposition of the great arteries and ventricular septal defect is feasible and should play an important part in the management of these patients.     

大鼠心肌肥厚时左心室心房钠尿肽基因的转录

本工作运用ANF放射免疫测定和ANF核酸分子杂交的方法,测定了大鼠腹主动脉部分结扎引起心肌肥厚时血浆ANF及左心室ANF mRNA水平的变化。结果表明,心肌肥厚大鼠的血浆ANF及左心室ANF mRNA水平都明显升高,提示大鼠心肌肥厚可以促进其左心室ANF基因的转录和表达。此外,本工作还证明细胞内钙离子调节剂牛磺酸和血管松弛剂肼苯哒嗪可以分别促进和抑制大鼠心肌肥厚诱导的左心室ANF基因的转录和表达。     

Polymorphic Ventricular Tachycardia Due to Acute Coronary Ischemia: A Case Report

Acute myocardial ischemia can cause ventricular tachycardia (VT) in patients with structurally normal heart. Contrary to the fact that in patients with chronic myocardial scarring the ventricular tachycardia is monomorphic, in patients with acute ischemia the ventricular tachycardia is polymorphic and is reversible with coronary revascularization.We are reporting a 40 year old male who presented with recurrent syncope due to polymorphic ventricular tachycardia in the context of normal QT interval in baseline ECG and normal left ventricular function without any evidence of myocardial injury. Due to recurrent fatal ventricular arrhythmia despite medical management, urgent coronary angiography was done which showed critical obstruction of right coronary artery (RCA). Considering the critical obstruction of RCA responsible for polymorphic VT, emergency PCI of RCA was done. After successful PTCA and stenting to RCA, he had another episode of polymorphic VT which was terminated with intravenous phenytoin. Seven days after the PCI, 24 hours Holter monitoring was done which showed normal sinus rhythm with infrequent ventricular premature complexes and no evidence of VT. He was asymptomatic at six months follow-up.     

Undiagnosed hypertrophic obstructive cardiomyopathy during transcatheter aortic valve replacement: a case report

Background

Transcatheter aortic valve replacement is indicated for severe symptomatic aortic stenosis in patients who have a very high or prohibitive surgical risk as assessed pre-procedurally by the Society of Thoracic Surgery Risk Score, EuroSCORE (II), frailty testing, and other predictors. When combined with another left ventricular outflow tract obstruction, careful consideration must be taken prior to proceeding with transcatheter aortic valve replacement because an additional masked left ventricular outflow tract pathology can lead to challenging hemodynamics in the peri-deployment phase, as reported in this case.

Case presentation

A 56-year-old Caucasian man with multiple comorbidities and severe aortic stenosis underwent transcatheter aortic valve replacement under monitored anesthesia care. During the deployment phase, he developed dyspnea that progressed to pulmonary edema requiring emergent conversion to general anesthesia, orotracheal intubation, acute respiratory distress syndrome-type ventilation, and vasopressor medications. Intraoperative transesophageal echocardiography was performed and hypertrophic obstructive cardiomyopathy with systolic anterior motion of the mitral valve was discovered as an underlying pathology, undetected on preoperative imaging. After treatment with beta blockers, fluid resuscitation, and alpha-1 agonists, he stabilized and was eventually discharged from our hospital without any lasting sequelae.

Conclusions

Patients with aortic stenosis most often develop symmetric hypertrophy; however, a small subset has asymmetric septal hypertrophy leading to left ventricular outflow tract obstruction. In cases of severe aortic stenosis, however, evidence of left ventricular outflow tract obstruction via both symptoms and echocardiographic findings may be minimized due to extremely high afterload on the left ventricle. Diagnosing a left ventricular outflow tract obstruction as the cause of hemodynamic instability during transcatheter aortic valve replacement, in the absence of abnormal findings on echocardiogram preoperatively, requires a high index of clinical suspicion. The management of acute onset left ventricular outflow tract obstruction intraoperatively consists primarily of medical therapy, including rate control, adequate volume resuscitation, and avoidance of inotropes. With persistently elevated gradients, interventional treatments may be considered.

     

猪冠状动脉的解剖学观察

本文对50例健康的商品猪心脏的铸型标本进行了观察,结果如下:左冠状动脉旋枝与锥旁室间枝的夹角为74.4±2.07度。对角枝出现率为24％,并证明了对角枝出现率与其夹角大小呈正比关系。窦房结枝84％来自右冠状动脉。房室结枝98％来自右冠状动脉。左房旋枝出现率为8％。室间隔的供血由锥旁室间枝的分枝负担61％,约为3/5,窦下室间枝的分技负担39％,约为2/5。室上嵴技、Kugel动脉、室间隔中枝和室间隔背倒前枝的出现率分别为78％、20％、68％和28％。心尖区的血液由左、右冠状动脉共同供应。50例左、右冠状动脉始部外径之比为1.2∶1。左、右冠状动脉在心膈面的分布类型以右强型为主。本文还讨论了猪冠状动脉与狗、人冠状动脉的异同。     

The state of the coronary arteries during balloon dilatation following thrombolytic therapy of acute myocardial infarction (a quantitative angiographic study)

The paper is concerned with the investigation of 52 patients who received i.v. thrombolytic therapy and "delayed" balloon dilatation of the coronary arteries in the first hours of acute myocardial infarction for the first 6 days. The investigation was repeated 6 mos after the onset of disease. Analysis of the coronaroventriculography results has shown that a combined use of i.v. thrombolytic therapy and delayed balloon dilatation of the coronary arteries results in a considerable and prolonged improvement of vascular permeability attended by a significant improvement of left ventricular local function and a decrease in the number of reocclusions. Infarction-related artery restenosis after combined therapy has little influence on left ventricular function which can be probably associated with a slow rate of its formation. Infarction-related vascular reocclusion leads to marked impairment of left ventricular total and local function.     

Congenital Aortic Stenosis: Some Observations on the Natural History and Clinical Assessment

Three hundred patients, 30 years of age or under, with the clinical diagnosis of aortic stenosis were reviewed to provide information on the accuracy of clinical assessment and the natural history of the condition when left untreated. Sudden death was uncommon and occurred only in patients with clinical evidence of severe obstruction. In infants, the early presentation and lethal nature of aortic stenosis appeared to result from the presence of additional cardiac lesions. Correlation of clinical assessment with hemodynamic data in 83 patients indicated that important stenosis was present if the systolic murmur was accompanied by a thrill and associated with an increased left ventricular impulse, decreased brachial artery pulse pressure, or left ventricular hypertrophy on the electrocardiogram. The site of obstruction could not be established with certainty by clinical examination, but an early systolic ejection click was strong evidence against subvalvular stenosis.     

Morphology of aortic arch obstruction with patent ductus arteriosus

Thirty-one hearts with aortic arch obstruction and patent ductus arteriosus were examined with special reference to associated cardiac anomalies. Six presented with complete interruption of the aortic arch, four with atretic isthmus, twelve with coarctation, and three with tubular hypoplasia. Associated cardiac anomalies were divided into two main groups: (1) septal defect with left-to-right shunt, and (2) left ventricular inflow and/or outflow obstruction. A high incidence (9/19=47.4%) of ventriculo-infundibular malalignment type of ventricular septal defect with subaortic stenosis was observed. Associated cardiac lesions that reduce blood flow in the aortic arch during fetal life may be responsible for poor development of this structure.     

Evaluation of ultrasound lung comets by hand-held echocardiography

Background

Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction. Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm. An even more rare presentation is the coexistece mid-ventricular and apical HCM. This case is a combination of obstructive HCM with mid-ventricular HCM and an apical aneurysm, which to date, has not been reported in the literature.

Case presentation

The patient is a 49 year-old lady who presents a combination of septal asymmetric hypertrophic cardiomyopathy (HCM) and midventricular HCM, a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, plus an apical aneurysm. Her clinical presentation was an acute myocardial infarction in June 2005. One month after hospital discharge, the electrocardiogram (ECG) showed a right bundle branch block (RBBB) with no Q waves or ST segment elevation. Coronary angiography revealed normal coronary arteries, left ventricular hypertrophy and an apical aneurysm.

Conclusion

This case is a rare example of an asymptomatic patient with subaortic and mid-ventricular hypertrophic cardiomyopathy, who presents a myocardial infarction and normal coronary arteries, and during the course of her disease develops an apical aneurysm.     

产前B 超诊断胎儿完全性大动脉转位的超声特征观察

目的:探讨产前B超对胎儿完全性大动脉转位的临床诊断价值。方法:回顾性分析2012年2月至2015年11月我院收治的4例胎儿完全性大动脉转位的B超特征,并对比病理结果。结果:4例完全性大动脉转位胎儿中,1例室间隔缺损,3例四腔心切面正常。左右两室流出道切面情况:4例胎儿大动脉与心室连接关系存在异常,2例胎儿室间隔膜部存在缺损症状。三血管气管切面情况:4例胎儿均仅可见2条血管。结论:对左右两心室流出道切面及三血管气管切面进行观察可得,胎儿完全性大动脉转位具有较为明显的B超特征,产前对胎儿完全性大动脉转位进行B超诊断具有较好的临床价值,建议在医疗单位推广应用。     

HYSTERICAL ABDOMINAL DISTENTION SIMULATING ACUTE INTESTINAL OBSTRUCTION: WITH REPORT OF A CASE

In hysterical abdominal distention the symptoms may so closely simulate those of obstruction of the bowel that needless laparotomy is carried out.Clues that indicate hysterical distention rather than organic cause are: (1) History of vomiting but without dehydration; (2) complaint of severe pain although temperature, pulse and the number of leukocytes in the blood all are normal; (3) normal tympanic quality of the abdomen; (4) distention out of proportion to the amount of gas observed in x-ray examination; (5) pronounced lordosis caused by thrusting the abdomen forward; and (6) remission of distention when the patient is under general anesthesia.It must be remembered, however, that even when there is strong suspicion of hysterical cause, distention may actually be due to obstruction. In the case herein reported, obstructive adhesion finally did occur after the patient had had numerous exploratory operations in which the viscera were observed to be normal.     

Coronary artery surgery.

Coronary artery disease has been described as the largest public health problem in Western society. In spite of the many advances in recent years in its medical management, many patients remain disabled even after optimal medical therapy. The aortocoronary bypass operation, introduced in the mid-1960s, has been shown to have consistent subjective and objective effects on the course of the disease in a large proportion of patients. The procedure consists of inserting a portion of the saphenous vein into both the aorta and a coronary artery to bypass the obstruction. It is usual now to bypass obstructions in several coronary arteries at the same operation if necessary. The prognosis for patients with ischemic heart disease with medical management depends on the extent of the disease. Patients with obstruction of only one coronary artery have a prognosis very little different from normal. On the other hand, obstruction of several arteries is consistently associated with a mortality approaching or exceeding 10% per year.