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1/14. Intraprocedural myocardial contrast echocardiography as a routine procedure in percutaneous transluminal septal myocardial ablation: detection of threatening myocardial necrosis distant from the septal target area.

    Percutaneous transluminal septal myocardial ablation (PTSMA) has been introduced as an alternative to surgery for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Visualization of the ablation area prior to induction of the chemical necrosis is possible by intraprocedural myocardial contrast echocardiography (MCE). We report on two patients in whom MCE showed opacification of the medial papillary muscle or the left ventricular posterolateral free wall. In both patients the correct ablation area could be identified by MCE after a change of the target vessel, thus avoiding potentially fatal complications due to induction of a necrosis of myocardium distant from the septal target area.
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2/14. Combination of aneurysm and myocardial bridging at the same site of a coronary artery in a patient with obstructive hypertrophic cardiomyopathy.

    This case report describes the rare finding of myocardial bridging and a coronary aneurysm in the same coronary artery segment of a 57-year-old patient with obstructive hypertrophic cardiomyopathy. At the site of the aneurysm in the proximal LAD, the myocardial bridging resulted in an almost normal vessel diameter during systole with an aneurysmatic expansion of the vessel during diastole. This accidental finding does not necessarily require special therapy, since the underlying coronary aneurysm is small, with a low risk of thrombus formation or rupture, but it is big enough to prevent a coronary obstruction due to the myocardial bridging.
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3/14. Usefulness of selective myocardial contrast echocardiography in percutaneous transluminal septal myocardial ablation: a case report.

    A 67-year-old woman with hypertrophic obstructive cardiomyopathy that was refractory to medical treatment underwent percutaneous transluminal septal myocardial ablation (PTSMA). The septal branch supplying the myocardium involved in the left ventricular outflow tract (LVOT) obstruction was identified by selective myocardial contrast echocardiography (MCE). MCE for the third and largest septal branch opacified the right side of the mid-septal myocardium and MCE for the second septal branch opacified the right side of the basal portion of the septal myocardium. Finally, contrast agent was injected into the first, small branch, which opacificied the myocardium protruding into the LVOT. Subsequently, septal myocardial ablation for this vessel with intracoronary alcohol was performed, followed by a reduction of the LVOT gradient and successful, dramatic improvement in the patient's clinical condition. Selective MCE was very useful to identify the appropriate septal branch for PTSMA and enabled maximal effect of this treatment with minimal myocardial damage.
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4/14. Apical hypertrophic cardiomyopathy: a case of slow flow in lad and malign ventricular arrhythmia.

    The coronary slow flow phenomenon is an angiographic finding characterized by delayed distal vessel opacification in the absence of epicardial coronary artery disease. patients often present with acute coronary syndrome. Histopathologic studies have revealed the existence of fibromuscular hyperplasia and myofibrilar hypertrophy. Apical hypertrophic cardiomyopathy is a benign progressive form of hypertrophic cardiomyopathy, that is rarely observed in western communities. It remains commonly asymptomatic until advanced ages. syncope, arrhythmia or sudden death may be the first symptom. We report a case of slow coronary arterial flow in a 71-year-old male patient with apical hypertrophic cardiomyopathy who experienced chest pain and sudden cardiac arrest due to ventricular arrhythmia.
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5/14. Tako-Tsubo cardiomyopathy: new insights into the possible underlying pathophysiology.

    Tako-Tsubo cardiomyopathy is characterised by an atypical distribution of left ventricular (LV) dysynergy with apical ballooning and compensatory basal hyperkinesis. coronary angiography is normal. Several substrates have been put forward to explain the underlying pathophysiology such as raised catecholamine levels (due to physical or emotional stress), multivessel epicardial coronary spasm or diffuse microvascular spasm. However, the pathophysiology has not yet been fully clarified. We present a series of cases whose findings could explain the mechanism underlying this syndrome. Four consecutives patients, all female, were admitted with the clinical features typical of Tako-Tsubo syndrome. In all, severe widespread transient LV mid-apical a/dyskinesia was associated with a mid-cavity dynamic obstruction which resolved prior to the resolution of the LV wall motion abnormalities. In all cases the dynamic LV obstruction was related to localised mid-ventricular septal thickening. After improvement in wall motion, a low-dose strain/strain rate dobutamine stress-echocardiography (DSE) was performed to determine the underlying ischaemic substrate. This provoked an LV mid-cavity gradient at peak dose in all. Regional deformation changes during DSE showed the affected myocardium to have the typical response diagnostic of regional stunning. CONCLUSION: We postulate that an important unrecognised factor in the development of Tako-Tsubo cardiomyopathy is the presence of abnormal myocardial functional architecture (such as localised mid-ventricular septal thickening), which in the presence of dehydration and/or raised catecholamine levels due to physical or emotional stress, leads the development of a severe transient LV mid-cavity obstruction. This effectively sub-divides the LV into two functionally different chambers with a marked increase in wall stress in the high pressure distal apical chamber. This, in combination with the abnormal high circulating catecholamine levels, induces widespread sub-endocardial ischaemia which is unrelated to a specific coronary artery territory. With rehydration/fall in catecholamine levels the interventricular gradient resolves and distal function recovers. Low dose SR/S DSE confirms that the distal ischaemic substrate is myocardial stunning.
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6/14. Esmolol (Brevibloc) to assess dynamic subpulmonary stenosis in a child after repair of complete transposition: a case report.

    Esmolol (Brevibloc), a new, ultra-short acting, cardioselective beta-adrenergic blocking agent with half-life of 9.2 min following i.v. administration was given to a 4-year-old child with known dynamic and fixed sub-pulmonary stenosis post Senning repair for complete transposition of the great vessels. The left ventricular systolic pressure increased from 48 to 100 mmHg, heart rate showed an increase from 65 to 140 bpm, the right femoral arterial pressures decreased from 115/58 to 77/35 mmHg following an infusion of Isoprel. Infusion of esmolol partially relieved the dynamic sub-pulmonary stenosis. There were no adverse effects and esmolol was tolerated well by the child. Esmolol might thus play a role in the pediatric catheterization laboratory during investigational procedures, electrophysiological studies and in the control of rapid supraventricular tachycardia, especially in adolescents with WPW pre-excitation. Esmolol would also be beneficial in emergency treatment of epinephrine or isoproterenol overdosage.
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7/14. Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration.

    The authors report a congenital portosystemic shunt which was detected with ultrasound in an infant with a fatal congenital hypertrophic cardiomyopathy. An aberration in primitive vitelline venous development may have contributed to the formation of this unusual vessel.
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8/14. Subaortic stenosis and sudden death.

    An obese white boy, 16 years old, collapsed and suddenly died while walking with friends. Since early childhood he had been known to have mild subaortic stenosis. At age 6 he exhibited first-degree heart block during an electrocardiographic exercise test. Neither then nor thereafter was he known to have any unusual symptoms until his death. Postmortem examination revealed marked cardiac enlargement without asymmetry or much dilatation. There was mild subaortic stenosis, but the heart was otherwise grossly normal. Special studies of his cardiac conduction system demonstrated fibrotic obliteration of the His bundle and proximal portions of both bundle branches. The same region had a much thickened central fibrous body from which the subaortic stenosis ridge protruded. Small arteries in the vicinity of the atrioventricular node were markedly narrowed. Because those vessels provide some of the blood supply to the His bundle region, their narrowing may have contributed to the fibrotic abnormalities observed. The extent of destruction of the His bundle makes a lethal failure of atrioventricular conduction the most likely terminal event.
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9/14. Single ventricle (single- or double-inlet) complicated by subaortic stenosis: surgical options in infancy.

    Intracardiac obstruction to the systemic circulation can develop in patients with many forms of congenital heart disease. When transposition of the great arteries accompanies tricuspid atresia, narrowing of the ventricular septal defect (VSD) leads to subaortic stenosis. In a similar fashion, a restrictive bulboventricular foramen compromises systemic arterial outflow in patients who have single left ventricle with subaortic outflow chamber. The same effect can be seen in VSD closure in mitral atresia with normally related great vessels. Although some forms of single ventricle can be treated by ventricular septation, the modified fontan procedure can be more generally applied to this entity. Pulmonary vascular resistance must be low, however, which excludes the application of the Fontan principle in infants. While pulmonary artery banding may protect the lungs from the development of excessive pulmonary vascular resistance, it may also accelerate the development of subaortic obstruction. We have successfully applied the Norwood operation, as described for hypoplastic left heart syndrome, to palliate subaortic stenosis due to restrictive VSD in 3 infants with single ventricle or atrioventricular valve atresia. Consideration should be given to a primary Norwood procedure in infants with single ventricle in whom subaortic stenosis is likely to develop. patients who do receive pulmonary artery bands should be followed closely for the development of subaortic stenosis and should undergo an early, physiologically corrective operation if it occurs.
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10/14. myocardial bridging and hypertrophic cardiomyopathy: relief of ischemia by surgery.

    We describe a case of myocardial bridging of the anterior descending coronary artery associated with a non-obstructive hypertrophic cardiomyopathy which was corrected by surgery because of a poor response to standard drug therapy. The clinical significance of the association is discussed. The possibility of repair by surgery should be considered when an area of ischemia supplied by the affected vessel is detected and previous medical treatment has been ineffective.
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