Double-outlet right ventricle with intact ventricular septum.

In the present case report, we describe a malformation consisting of a double-outlet right ventricle with an intact ventricular septum diagnosed in a 3-day-old female newborn. To our knowledge 35 cases have been described in the literature. The diagnosis was made by two-dimensional echocardiography and confirmed by angiocardiography. An inadequate opening in the interatrial septum and hypoplasia of the mitral valve and left ventricle were present. In this condition the only outlet of the left ventricle is via the atrial septal defect. Balloon atrial septostomy was performed. At 1 month of age the patient required a right modified Blalock-Taussig shunt and surgical atrial septectomy. Five months after surgery, the patient was in good clinical conditions.

Single coronary artery: a case report.

We report a case of a 47-year-old woman with a previous myocardial infarction, in whom coronary angiography showed a right-type single coronary artery (arising from a single ostium in the right sinus of Valsalva) without associated obstructive lesions. We have demonstrated that the transesophageal echocardiogram was complementary to angiography in the assessment of retroaortic course of the anomalous left main coronary artery.

[Asymptomatic left ventricular myxoma in pregnancy: echocardiographic diagnosis and surgical treatment]. / Mixoma ventricolare sinistro asintomatico in gravidanza: diagnosi ecocardiografica e terapia chirurgica.

Myxoma arising from the endocardial wall of the ventricles is rare. We describe here the case of a young woman in whom an asymptomatic left ventricular myxoma was incidentally discovered at the 35th week of pregnancy by transthoracic echocardiography. The echocardiogram revealed a huge intraventricular mass attached to the apex and prolapsing to the aortic valve during systole. On the basis of this ominous feature, the woman was promptly submitted to caesarean section and, after 10 days, to surgical removal of the mass.

[The isolated agenesis of a branch of the pulmonary artery. A case report: its diagnosis and therapy]. / Agenesia isolata di un ramo dell'arteria polmonare. Presentazione di un caso: diagnosi e terapia.

We illustrate a case of so-called right pulmonary artery agenesis, discovered in a 3-month-old baby. This kind of malformation is rarely reported in neonatal age being most often discovered in the adulthood. The patient presented with cardiac failure, cyanosis and radiological features of cardiomegaly and left side pulmonary venous congestion. The diagnosis was strongly suspected by two-dimensional echocardiography and confirmed at cardiac catheterization. The patient was successfully operated upon with connection of main pulmonary artery to hilar right pulmonary artery. To the best of our knowledge, this is the first reported case of diagnosis and effective surgical therapy of right pulmonary artery agenesis in neonatal age.

[Perfusion catheter and cardio-pulmonary support standby: organizational strategy for high risk PTCA. Description of a case treated successfully]. / Catetere perfusore e supporto cardio-polmonare standby: strategia organizzativa per PTCA ad alto rischio. Descrizione di un caso tentato con successo.

The organizational strategy used in a high risk Coronary Angioplasty procedure is discussed. The attempted vessel was a severely stenosed Right Coronary Artery in a patient who had undergone triple graft, with residual obstruction of Left Main Artery and only one patent venous graft on a marginal branch of the Circumflex artery. The procedure was performed using a Stack type autoperfusion balloon angioplasty catheter, with a Cardiopulmonary Support System held in standby.

["Kissing balloon" of the protected common trunk of the left coronary artery: description of a successfully treated case]. / "Kissing balloon" del tronco comune della coronaria sinistra protetto: descrizione di un caso trattato con successo.

The authors describe a case of coronary angioplasty on a distal Left Main coronary lesion. The left coronary tree was protected by the left internal mammary connected to left anterior descending artery. The procedure was performed using the "kissing balloon" technique, with a single guiding catheter. Immediate angiographic result and one-year follow-up have been excellent.

[Coronary angioplasty in patients with compromised left ventricular function]. / Angioplastica coronarica in pazienti con funzione ventricolare sinistra compromessa.

One thousand one hundred and thirty-two percutaneous transluminal coronary angioplasties have been performed at Centro Cuore Columbus in Milan between January 1987 and September 1989. The whole population was divided into two groups: Group A (90 patients with ejection fraction less than or equal to 0.40); Group B (678 patients with ejection fraction greater than 0.40. Mean ejection fraction in Group A (0.35) was significantly lower (p less than 0.0001) than in Group B (0.56). In each group three subsets were identified: subgroup 1: total obstruction of two major epicardial vessels plus severe stenosis of the patent one; subgroup 2: total obstruction of one vessel plus severe stenosis on a different remote one; subgroup 3: all patent vessels with one or more severe stenoses on one or all. We attempted percutaneous transluminal coronary angioplasties on 923 stenosed and 161 totally obstructed vessels, with a primary success of 95 and 56% respectively. We experienced 36 major complications (7 deaths, 10 acute myocardial infarctions, 19 emergency aortocoronary bypasses in 21 patients (2.7% of the whole population). Subgroup A1 (7 patients with ejection fraction less than or equal to 0.40 and attempted percutaneous transluminal coronary angioplasties on the sole patent vessel) showed the lowest success rate (44%) and the highest complication rate: 3 deaths (42.8%); 1 acute myocardial infarction (14.2%); 3 emergency aorto-coronary by-pass (42.8%). The 83 patients in group A2 and A3, with reduced ejection fraction, did not have significantly higher complication rate than all group B patients, with normal ejection fraction.(ABSTRACT TRUNCATED AT 250 WORDS)

["Myocardial bridge" and "intramural LAD pathway" are not just a semantic problem]. / "Ponte miocardico" e "decorso intramurale dell'IVA" non solo un problema semantico.

The angiographic definition of "Myocardial Bridge" universally applies to signify systolic narrowing of a coronary segment by strips of myocardial tissue. The Authors report the clinical history of two patients suffering from effort angina and transient myocardial ischemia, in whom coronary angiography showed no atherosclerotic lesions, but wide systolic kinking of the left anterior descending (LAD) artery, without systolic luminal obstruction. The definition of "Intramural LAD Pathway" has been put off in place of "Myocardial Bridge", making a distinction not simply semantic but between two different angiographic and pathologic pictures. It is very likely indeed that coronary flow impairment, solely systolic in myocardial bridge, takes places mainly during diastole in Intramural LAD Pathway.

[Multiple coronary artery-left ventricle fistulas in a patient with apical hypertrophic cardiomyopathy: an unusual cause of angina pectoris]. / Fistole multiple coronaria-ventricolo sinistro in paziente con cardiomiopatia ipertrofica apicale: causa insolita di angina pectoris.

A 50 year-old man had a history of crescendo exertional angina. Echocardiographic and electrocardiographic findings were typical of apical hypertrophic cardiomyopathy. Selective coronary angiography showed no atherosclerotic lesions, but there was a stream of dye into the left ventricle, via a tiny plexus of intramural vessels, from the distal third of the anterior descending coronary artery. The origin of myocardial ischemia has been related to a "steal" phenomenon, while this vascular anomaly could represent a disorder of the thebesian system, possibly due to the "disarray" of myocardial cells typical of hypertrophic cardiomyopathy.

[Idiopathic dilatation of the atria: a syndrome? Description of a case of idiopathic biatrial dilatation, review of the literature and a proposal for a pathogenetic interpretation]. / La dilatazione idiopatica degli atri: una sindrome? Descrizione di un caso di dilatazione idiopatica biatriale, revisione della letteratura e proposta di interpretazione patogenetica.

Idiopathic severe dilatation of bilateral atrial and urethero-pelvic systems was found in a 53-year-old woman. Degeneration of muscle fibers without inflammation is a constant feature of idiopathic atrial enlargement. Degenerative process can extensively involve atrial wall (generalized dilatation) or can be localized (aneurysmal dilatation). In both types of lesion clinical features are related to atrial arrhythmias and to compression on atrio-ventricular valves and ventricles by the enlarged atria. Only the outcome of the atrial arrhythmias can help to distinguish between the two conditions: development of irreversible atrial standstill in generalized dilatation and definitive restoration of sinus rhythm, following surgery, in aneurysmal dilatation. In each atrium, a different rate of incidence of the two types of dilatation has been documented in the literature: as for the right atrium, more than 40 cases of generalized dilatation have been reported and only 4 of the aneurysmal type. On the other hand, isolated left atrial enlargement was of aneurysmal type in all 30 reported cases. Biatrial dilatation was generalized in 7 cases and due to multiple aneurysms in a single case. The Authors support the hypothesis that idiopathic atrial dilatation could be due to a degenerative process affecting the atrial components derived from primitive atria, in which the muscular layer is structured into pectinate muscles. They also suggest to distinguish, even from the etiopathogenetic point of view, between aneurysmal dilatation (localized and fortuitous lesion) and generalized dilatation (diffuse degeneration of pectinate muscles). This distinction could well related with the different outcome of atrial arrhythmia in the two types of dilatation.(ABSTRACT TRUNCATED AT 250 WORDS)

[Aortic insufficiency caused by valvular dysplasia in childhood. Apropos of 3 cases]. / Insufficienza aortica da displasia valvolare nella fanciullezza. A proposito di tre casi osservati.

Three patients whose aortic incompetence (A.I.) was recognized in childhood, have had an angiographic study 10, 9 and 4 years, respectively, following the first clinical diagnosis of their valvular disease. A.I. was related, in all three cases, to a dysplastic valve ("floppy valve syndrome"); in none of them was there an abnormal dilatation of ascending aorta. In our opinion, the absence of an abnormal aortic root dilatation separates our cases from those in whom aortic regurgitation is part of Marfan's syndrome, either in its full form or in the "forme fruste". From the clinical viewpoint, the presence of a normal ascending aorta could explain the mild course of these cases, as compared to the rapid progression observed in Marfan's syndrome. In the attempt to differentiate the two types of dysplastic valvular disease from an etiologic viewpoint, we suggest that the basic problem in our patients could be an alteration of the valvular connective tissue development, rather than a genetic disorder, like the one possibly responsible for Marfan's syndrome.

Two-dimensional echocardiographic assessment of surgically closed ventricular septal defect.

One hundred twenty patients operated on for closure of a ventricular septal defect (VSD) had postoperative assessment by 2-dimensional echocardiography. The presence of persistent "drop-out" between ventricular septum (VS) and the periphery of the "patch" was considered a sign of detachment and residual shunt. Echocardiographic findings of intact VS or residual VSD were correlated with angiographic findings in 30 patients and with clinical signs in 90 patients. Minute shunts with increased flow ratios less than 1.5:1 were occasionally missed. Persistent small drop-out was sometimes wrongly diagnosed as residual shunt when the patch was in an angled position and in the early postoperative weeks when peripatch edema of the VS suggested drop-out. Thus, 2-dimensional echocardiography is a reliable method for assessing significant persistent ventricular shunts and intact VS after surgical closure of a VSD.

Aorto-left ventricular communication after closure. Late postoperative problems.

The long-term follow-up of six patients operated on for aorto-left ventricular communication has been reviewed in detail. All had residual aortic regurgitation after the initial repair of the defect. It was severe in four and required repeated reoperation in three with ultimate aortic valve replacement. The failure of early repair to solve the haemodynamic problem has provoked a reconsideration of the basic anatomy, of the surgical approach, and of the postoperative physiology of this anomaly. The so called "tunnel" is not a tunnel with length but should be considered as a localised breach at the insertion of the right coronary cusp. The localised aortic root dilatation at the site is a weakness that remains after closure of the tunnel leaving a poorly supported aortic valve and a weak root. Thus, the initial repair of the aorto-left ventricular communication must not only close the communication but reinforce, strengthen, and support the right aortic sinus in order to maintain cusp competence.