[Infant case and adult case of scimitar syndrome with lung sequestration].

We present 2 cases of Scimitar syndrome with lung sequestration, 1 of which is infant case and the other is adult. A 3-month-old baby was transferred to our hospital because of severe heart failure. Cardiac ultrasonography and angiography revealed atrial septal defect and lung sequestration. Because of critical condition, she underwent emergency operation. After the operation of abnormal lung resection, her general condition improved. We emphasize that the prognosis is influenced by operation timing. A 20-year-old female was admitted for atrial septal defect. We diagnosed Scimitar syndrome and lung sequestration as the result of close examinations. We carried out definitive operations of the closure of atrial septal defect, the correction of partially anomalous pulmonary venous return and the resection of malformed lung. This adult case is popular one of Scimitar syndrome.

[Familial aortic dissection; cases involving a father, mother, and son].

The occurrence of familial aortic dissection is rare in cases that are not linked to Marfan syndrome. We report the 3 cases of acute aortic dissection, involving both parents and their son. Case 1 (father case): 79-year-old male visited a nearby doctor complaining of severe chest and back pain. He was diagnosed as acute aortic dissection (Stanford type A). An emergency operation was carried out and he had an artificial vascular replacement of ascending aorta and arch portion. Case 2 (mother case): 73-year-old female was treated for hypertension. Suddenly, she had a terrible epigastralgia and back pain. The contrast enhanced chest computed tomography (CT) revealed an acute aortic dissection (Stanford type A). The ascending aortic aneurysm ruptured in the ambulance while she was transferred to our hospital. She arrived at our hospital after cardiac and respiratory arrest. We attempted to perform cardiac massage, but we could not bring her back to life. Case 3 (son case): 48-year-old male was transferred to our hospital diagnosed with acute aortic dissection (Stanford type A). The ascending aorta replacement was performed on the same day. We were able to save cases 1 and 3 by performing surgery. Aortic dissection is thought to be the disease of medial degeneration. Hereditary links to aortic dissection are not well understood, with the exception of Marfan syndrome cases. The investigation of genetic mechanisms related to aortic dissection will be expected in the future.

[A difficult case of antiphospholipid syndrome with repeated restenosis of coronary artery and grafts].

A 65-year-old man complained of exertional angina. Coronary angiography revealed 99% stenosis in the left anterior descending artery (LAD). He underwent percutaneous transluminal coronary angioplasty (PTCA) and stenting. Repeat angiography demonstrated restenosis of the previous PTCA and stenting site. He underwent coronary artery bypass grafting (CABG) with placement of the left internal thoracic artery (LITA) to LAD. Chest discomfort and V1-V3 ST elevation appeared on the first post operative day. Coronary angiography revealed occlusion of the LITA graft. He underwent re-operation. Because the radial artery was severely sclerotic, the great saphenous vein was used for the graft. Two weeks later, he began to show edema in the left lower extremity. Echoangiogram showed occlusion of the left deep vein with thrombus. He tested positive for anticardiolipin IgG and IgM antibodies. Eighteen months after re-operation, he had recurrent chest discomfort on exertion. Coronary angiography revealed 90% stenosis of the anastomosis (SVG-LAD). A second re-operation was performed. We used the right internal thoracic artery (RITA) for the graft. The postoperative angiography showed patent graft. The patient has been doing well without any complications.

[MIDCAB for redo patients using the LITA-RA composite graft].

Redo coronary artery bypass grafting (CABG) is associated with higher mortality, low-output syndrome, perioperative myocardial infarction than primary CABG. Minimally invasive direct coronary artery bypass grafting (MIDCAB) technique avoids the manipulation of old graft and injury of the adhesive heart in redo operation. We performed the MIDCAB procedure for 2 redo cases using the left internal thoracic artery (LITA)-radial artery (RA) composite graft. The LITA-RA composite graft was anastomosed to the left anterior descending branch (LAD) through small left anterior thoracotomy without cardiopulmonary bypass. Postoperative coronary artery graphy shows the widely patent of new graft. The MIDCAB procedure using the LITA-RA composite graft is safe and useful to regulate the bypass graft length and avoid the widely harvest of LITA in redo operation.

[Two cases of stent graft repair for thoracic aortic dissection: usefulness of three-dimensional computed tomogram].

We carried out stent graft repair in two patients with Stanford type B thoracic aortic dissection. A 51-year-old male was admitted to our hospital because of thoracic aortic dissection. Chest CT revealed an aneurysm of the distal aortic arch. The entry was pointed out 1 cm distal from the take off of the left subclavian artery in three-dimensional CT (3 D-CT). He was treated with a Gianturco stent which was anchored into the 30 mm Hemashield graft under selective cerebral perfusion. Another case was a 72-year-old male with a descending aortic aneurysm. 3 D-CT showed that the entry existed 4 cm proximal to the celiac artery. We performed transluminal implantation of the spiral Z-stent covered with the woven Dacron graft. 3 D-CT was useful for the preoperative management and the surgical treatment of thoracic aortic dissection.

Evaluation of the internal thoracic arterial graft patency by the transthoracic Doppler method under continuous intravenous infusion of adenosine triphosphate disodium.

Usefulness of the Doppler method under continuous infusion of adenosine triphosphate disodium (ATP) for improvement of accuracy in the diagnosis of the left internal thoracic arterial graft (LITA) patency was examined using transthoracic ultrasonic echocardiography. 1) Influence of ATP on the Doppler velocity in a graft was examined in 7 patients with good LITA grafts using physiological saline as the control. In the ATP group, 80 mg of ATP was dissolved in 20 ml physiological saline and continuously infused at 0.14 mg/kg/min. In the saline group, an equal volume of physiological saline was administered and the blood flow velocity in the LITA was recorded continuously by the transthoracic Doppler method from the supraclavicular fossa approach. Results; ATP administration increased the blood flow velocity in the LITA and the rate of increase was 48.3% for systolic peak velocity, 111% for diastolic peak velocity, 64.4% for systolic time velocity integral and 99% for diastolic time velocity integral indicating particularly high rates of increase in diastolic components. The diastolic/systolic peak velocity ratio or diastolic fraction did not increase significantly. In the saline group, none of the parameters showed a change. 2) Angiographic findings of the LITA were compared with the measurement values of the diastolic components by the Doppler method to examine usefulness of diastolic component measurement with ATP infusion for diagnosis of LITA patency. Subjects were 19 patients with good LITA (group A) and 8 patients with bad LITA (group B). Results; while there were significant differences in the mean baseline diastolic peak velocity, mean diastolic time velocity integral and mean diastolic fraction between the groups, overlapping was seen in individual cases. However, the inter-group differences were more distinct by ATP infusion and the borderline values were 30 cm/sec for diastolic peak velocity and 10 for diastolic time velocity integral. 3) Reliability of the diagnosis for LITA patency by measuring the diastolic components using the Doppler method with ATP infusion was examined and compared with the angiographic findings as the gold standard. Subjects were 27 patients and the diagnostic criteria for good LITA were set at 30 cm/sec for diastolic peak velocity and 10 for diastolic time velocity integral. Results; sensitivity and specificity of the Doppler method with ATP infusion were 100% for diagnosis of LITA patency by measuring the diastolic components. Conclusion, in diagnosis of LITA patency by the transthoracic ultrasonic cardiography, diagnostic accuracy was improved by measuring the diastolic parameters under continuous infusion of ATP.

Effect of amiloride on ischaemia and reperfusion injury in isolated, perfused rat hearts.

The effect of amiloride, a potent inhibitor of Na+/H+ exchange, on ischaemic reperfused rat hearts was studied in order to investigate whether Na+/H+ exchange or Na+/Ca2+ exchange is involved in ischaemia-reperfusion injury, When hearts were pre-ischaemically loaded with 100 microM amiloride, recovery of left ventricular developed pressure was significantly better than in control hearts, whereas recovery of heart rate at 30-min reperfusion was unaffected. Amiloride pretreatment also decreased creatine phosphokinase activity in the coronary effluent and completely abolished occurrence of ventricular arrhythmias during reperfusion. It also inhibited intracellular Na+ accumulation early in reperfusion (within 5 min), whereas in the late stage (from 5 to 30 min), Ca2+ overload was inhibited. The findings suggest that Na+/H+ exchange participates mainly in the early stage of reperfusion injury and the Na+/Ca2+ exchange system, secondary to Na+/H+ exchange, in the late stage. The reduction in post-ischaemic cardiac dysfunction induced by amiloride pretreatment may be attributable to inhibition of the resultant Ca2+ accumulation during reperfusion.

Atrial natriuretic peptide stimulates Na+-dependent Ca2+ efflux from freshly isolated adult rat cardiomyocytes.

In the present study, we examined the effect of atrial natriuretic peptide (ANP) on Ca2+ efflux from freshly isolated adult rat cardiomyocytes. Rat ANP(1-28) stimulated the efflux of 45Ca2+ from the cells in a concentration-dependent manner (10(-8) M to 10(-6) M). The 45Ca2+ efflux was not affected by removal of extracellular Ca2+, but was dependent on the presence of extracellular Na+. In addition, rat ANP(1-28) caused 22Na+ influx into the cells. The 45Ca2+ efflux was also stimulated by C-type natriuretic peptide-22 (CNP-22), but not by rat brain natriuretic peptide-45 (BNP-45). It was also observed that both rat ANP(1-28) and CNP-22 stimulated guanosine 3',5'-cyclic monophosphate production within the cells. These results indicate that ANP stimulates Na+-dependent 45Ca2+ efflux from freshly isolated adult rat cardiomyocytes, probably through Na+/Ca2+ exchange, and that the stimulatory effect of ANP on Ca2+ efflux may be mediated via the natriuretic peptide receptor which has been shown to couple to guanylate cyclase. Since it is reported that Na+/Ca2+ exchange is important in calcium homeostasis within cells, ANP may play a role in the extrusion of intracellular Ca2+ from isolated adult rat cardiomyocytes.

Neuropeptide Y as a stimulator of Na+ -dependent Ca2+ efflux from freshly isolated adult rat cardiomyocytes.

Several physiological stimuli cause a rise in intracellular Ca2+ concentration ([Ca2+]i) in cardiomyocytes. This increased [Ca2+]i must be restored to physiological resting level to ensure response to further stimuli. In the present study, we examined the effect of neuropeptide Y (NPY), which is secreted from certain adrenergic or nonadrenergic neurons, on Ca2+ efflux from freshly isolated, quiescent adult rat cardiomyocytes. The isolated cardiomyocytes were preloaded with 45CaCl2 for 1 h. Then, the fractional release of 45Ca2+ from the cells was measured. NPY stimulated the efflux of 45Ca2+ from isolated adult rat cardiomyocytes in a concentration-dependent manner (10(-8) M to 10(-6) M). NPY (10(-6) M)-induced Ca2+ efflux was 2.0 +/- 0.16% of the total cellular content. The 45Ca2+ efflux from the cells was also stimulated by Y1 receptor agonist, [Leu31, Pro34]NPY, but not by Y2 receptor agonist, NPY13-36. The effect of NPY was inhibited by a peptide NPY inhibitor, NPY18-36 and a non-peptide NPY inhibitor, benextramine to a similar extent. From these results, it is conceivable that the effect of NPY on Ca2+ efflux from cardiomyocytes is mediated through Y1 receptors. It was also observed that NPY caused a rise in [Ca2+]i to almost 150 nM. NPY-stimulated 45Ca2+ efflux was not affected by removal of extracellular Ca2+, but was dependent on the presence of extracellular Na+. Moreover, NPY caused a 22Na+ influx into the cells of about 1.6-fold over the basal value which was inhibited by amiloride and 5-(N,N-dimethyl)-amiloride, known Na+/Ca2+ exchange inhibitors. In addition, isoproterenol also caused 45Ca2+ efflux from the cells and which was enhanced by the addition of NPY. These results suggest that NPY stimulates extracellular Na+-dependent 45Ca2+ efflux from freshly isolated adult rat cardiomyocytes, probably through its stimulatory effect on plasma membrane Y1 receptors with which NPY may couple during Na+/Ca2+ exchange.

Newborn with congenital arteriovenous fistulas of the abdominal wall complicated by a ductus arteriosus aneurysm.

A case of congestive heart failure in a neonate resulting from a congenital abdominal arteriovenous fistula is described; an aneurysm of the ductus arteriosus was also found in the patient. Abdominal aortography was performed and demonstrated dilated feeding arteries, including the hypogastric arteries, which communicated with a dilated umbilical vein. Ligation of the feeding arteries and the umbilical vein led to resolution of the heart failure. Transient liver dysfunction occurred, however. Symptoms improved dramatically upon removal of the ligature from the umbilical vein. Although there is one previous report of an arteriovenous fistula involving the umbilical vein, we know of no prior report of a congenital arteriovenous fistula in association with a ductus arteriosus aneurysm.

[A rare complication of catheter balloon valvuloplasty of pulmonary stenosis--a case report].

We describe a rare complication which occurred during catheter balloon valvuloplasty to the stenosis of the pulmonary artery. A 4-year-old girl with cyanosis who was diagnosed as having tetralogy of Fallot, patent foramen ovale and Down's syndrome underwent the cardiac catheralisation and angiography. Angiography showed the stenosis from outlet of right ventricle to bifurcation of the pulmonary artery. We attempted catheter balloon valvuloplasty to the stenosis of the pulmonary artery subsequently. We used the PTA catheter (NuMED INC, canada), 10 mm in diameter. The balloon was inflated twice (6 atm, 5 sec, 8 atm, 7 sec) at the bifurcation of the pulmonary artery safely. However, during third trial to inflate to 8 atm, it proved impossible to increase pressure to the desired level of 8 atm. Blood backed up in the catheter and it became impossible to deflate the balloon. We failed to remove the catheter through the left femoral vein, so we led the catheter to the right internal jugular vein with a guidewire. The catheter was successfully extracted in this way. The proximal connection between the balloon and the outer tube was broken and the balloon was compressed. To our knowledge, such as complication as this has not reported previously.

New operative method for tracheal compression in congenital heart disease.

A new technique is described for repairing tracheal compression caused by ascending aorta associated with corrected transposition of the great arteries and pulmonary atresia in a 113-day-old infant. Surgical repair for the tracheal compression was accomplished by suspending the pericardium around the aortic arch to the underside of the sternum. This method seems to be useful for some patients with tracheal compression caused by the ascending aorta.

[A case of thoracic aortic aneurysm associated with atypical coarctation and kinking of the aortic arch].

The aneurysm associated with atypical coarctation and kinking of the aortic arch is rare. Authors experienced such a case and performed operation using bypass technique as below. A 17-year-old male admitted to the authors' hospital to undergo operation of the enlarging thoracic aneurysm. He was observed from his childhood diagnosed as the coarctation and kinking of the aortic arch. The chest X-ray films showed a mass shadow in the left superior mediastinum. It had been also diagnosed as having a small post-stenotic dilation of aortic arch. Recently the mass on the X-ray enlarged. He was diagnosed as an aneurysm located on aortic arch using MR-imaging and aortogram. The aneurysmectomy was performed because of the possibility of the aneurysmal rupture. Under the general anesthesia, extra-anatomical bypass was made between ascending aorta and descending aorta, and another one between the bypass and left common carotid like as T-shape, because the proximal aneurysm was very near located on the origin of left common carotid. The aneurysm was excised and woven Dacron graft was implanted. The post-operative course was uneventful. This case suggests the necessity of deliberate clinical observation using roentgenography for a case of kinking of the aortic arch.

[A case report of cor triatriatum benefit of MRI for preoperative diagnosis and surgical method].

Cor triatriatum is uncommon in all congenital heart diseases. It is a malformation resulting in a separation of the left atrium or right atrium into two chambers due to a congenitally abnormal diaphragm. We wish to present a case of cor triatriatum in which MRI was found most useful for preoperative diagnosis and surgical procedure. A 2-year-old girl was transferred to us for severe pulmonary congestion as shown on chest X-ray. Echocardiography showed abnormal diaphragm in the left atrium. MRI demonstrated clearly the relationship between left pulmonary vein and the abnormal diaphragm. Therefore we should preoperatively determine type I A according to the Lucas and Schmidt's classification. Cardiac catheterization showed moderate pulmonary hypertension and confirmed cor triatriatum. The resection of the abnormal diaphragm was performed under extracorporeal circulation with moderate hypothermia. The postoperative course was uneventful. MRI is a very useful non-invasive technique in making a diagnosis and in choosing the appropriate surgical procedure for cor triatriatum.

[Expression of atrial natriuretic polypeptide in ventricles of heart with hypertrophic cardiomyopathy: an immunohistochemical study of endomyocardial specimens].

To investigate the ventricular expression of atrial natriuretic polypeptide (ANP) in human hypertrophic heart, we conducted an immunohistochemical study using endomyocardial biopsy specimens obtained from the right side of the interventricular septum (RVB), left ventricular free wall (LVB), or both of 39 patients with hypertrophic cardiomyopathy (HCM), and 9 control subjects without hypertrophy. No HCM patients had apparent congestive heart failure. ANP was not present in control subjects' RVB or LVB specimens, but was found in HCM patients', showing its characteristic distribution patterns (RVB > LVB, p < 0.05); it was present in 15 of 36 RVB (42%) and 2 of 25 LVB (8%). No clinical data, including echocardiographic, hemodynamic and angiographic data, were directly related to ventricular ANP expression in HCM. According to histological data, however, ANP-present RVB specimens of HCM had larger myocytes, severer fibrosis and myofiber disarray than the specimens without ANP. This indicates that a failing state may not be a prerequisite for ANP expression in human hypertrophic ventricles, but that ventricular ANP expression may occur concomitantly with myocyte hypertrophy as an adaptive response to focal stress due to "histological overloads" such as disarray and fibrosis in HCM, which may be reflected in the characteristic distribution patterns of intraventricular ANP.

Relation between myoglobin and cardiac dysfunction in myocarditis--immunohistochemical study of endomyocardial biopsy specimens.

To investigate the mechanism of cardiac dysfunction in myocarditis, myoglobin, an intracellular oxygen-transport, was immunohistochemically examined in biopsy specimens obtained from the right side of the ventricular septum and left ventricular free wall in 58 patients with myocarditis and 19 controls. Sections 4 microns thick were stained by the indirect immunoperoxidase method using a polyclonal antibody to human myoglobin as the primary antibody. Under light microscopy, the intensity of myoglobin immunoreactivity in the tissue section was semiquantitatively classified from grade 0 to grade 3. Then, the grade of myoglobin staining was compared with clinical, hemodynamic and histopathologic parameters. In right and left ventricular specimens, the grade of myoglobin staining was positively correlated with ejection fraction, but inversely with left ventricular end-diastolic and end-systolic volume indices. The percentage of myocytes with grade 0 was correlated with the number of mononuclear cells in the specimens. In addition, the grade of myoglobin staining in right ventricular specimens was positively correlated with the duration of illness but inversely correlated with the number of mononuclear cells. In 4 patients who had serial biopsies, the ejection fraction was improved and the grade of myoglobin staining was increased in the convalescent stage. These results indicate that myoglobin staining reflects the intensity of myocarditis and a decrease of myoglobin may be important as one of the pathogenetic factors of cardiac dysfunction in myocarditis.

Ventricular expression of atrial natriuretic polypeptide and its relations with hemodynamics and histology in dilated human hearts. Immunohistochemical study of the endomyocardial biopsy specimens.

To investigate the mechanism of expression of atrial natriuretic polypeptide (ANP) in human ventricles, we conducted an immunohistochemical study of ANP in biventricular endomyocardial biopsy specimens obtained from a total of 49 patients with cardiac dilatation due to dilated cardiomyopathy (21 patients), postmyocarditis (18 patients), or volume overload (five patients) and subjects with no dilatation as controls (five patients). Four-micron thick sections were stained by an indirect immunoperoxidase method using monoclonal antibody to alpha-human ANP as the primary antibody. The frequency of ANP-present myocytes was calculated in each specimen and compared with clinical, echocardiographic, hemodynamic, angiographic, and histologic parameters. ANP-present myocytes were noted in all of the 21 patients with dilated cardiomyopathy, in 11 of the 18 patients with postmyocarditis, in four of the five patients with volume overload, and in zero of the five controls. The mean percentage of ANP-present myocytes was significantly greater in the left-side specimens (35 +/- 37%) than in the right-side ones (2 +/- 4%). The percentage of ANP-present myocytes in the left-side specimens significantly correlated with peak systolic or end-diastolic wall stress (r = 0.67 and 0.58), left ventricular end-systolic or end-diastolic volume index (r = 0.75 and 0.69), or left ventricular end-diastolic pressure (r = 0.42) and inversely correlated with ejection fraction (r = -0.73), systolic left ventricular wall thickness (r = -0.58), or cardiac index (r = -0.30). Expression of ANP was rarely seen in the cases with normal wall stresses, normal ejection fraction, normal volume, or normal myocyte size. However, it was seen frequently even in hearts with normal levels of left ventricular end-diastolic pressure and cardiac index (compensated hearts). The percent of ANP-present myocytes in both sides significantly correlated with size of myocytes (r = 0.48 at right and r = 0.57 at left side) or degree of fibrosis (r = 0.45 at right and r = 0.48 at left side). These results suggest that ANP expression is augmented in the dilated ventricles regardless of the causes of dilatation and that the augmentation is a compensatory mechanism as prevention against decompensation responding to reduced contractility, excess of wall stresses, or both, concomitantly occurring with cardiac dilatation and myocardial hypertrophy.

Infarct size and the protection of ischemic myocardium in pig, dog and human.

To define whether recanalization after occlusion can reduce the myocardial infarct size, we compared the infarct size in 25 pig hearts without collateral circulation, 35 dog hearts with collateral circulation and 11 human autopsied hearts with coronary thrombolysis at 2 to 6 hours after the onset of acute myocardial infarction. The data showed that % infarct size in the risk area increased according to the duration of occlusion. In the pig, % infarct size was 80 +/- 9% in the recanalization after 1 hour occlusion and 96 +/- 2% in the recanalization after 2 hour occlusion. There was no significant difference between these and the permanent occlusion group (95 +/- 3%). In the dog, % infarct size was 35 +/- 31% in the recanalization after 4 hour occlusion and 59 +/- 27% in the permanent occlusion group. In human autopsied hearts, the infarct size was the same between the recanalization group (82 +/- 6%) and the permanent occlusion group (80 +/- 11%). The % infarct size in the recanalization groups was less than or the same as that in the hearts with permanent occlusion in dog, pig and human. Thus, it is concluded that, to reduce conclusively the infarct size, recanalization should be done within 1 hour after the occlusion in the hearts without collateral circulation and within 4 hours in the hearts with collateral circulation. So called reperfusion injury which means the greater expansion of the % infarct size than that in the permanent occlusion is not present.