[Supravalvular aortic stenosis. Report of 3 cases]. / Stenosi sopravalvolare aortica. Descrizione di tre casi.

In the following article three cases of supravalvular aortic stenosis are presented: the first two cases refer to two brothers. The older, a 22-year-old man presenting with palpitations, underwent echocardiography and Doppler that showed an hour-glass supravalvular aortic stenosis with a peak gradient of 115 mmHg, associated with dilation of the left main coronary artery and stenosis of the left carotid artery at its origin. The patient's family was evaluated by echocardiography, and an 18-year-old brother was similarly found to have an hour-glass supravalvular aortic stenosis, graded mild to moderate (peak gradient 40 mmHg). Both cases are probably familiar forms of supravalvular aortic stenosis with normal facies and intelligence (autosomal dominant transmission). The elder brother, with severe stenosis, underwent surgical replacement of the ascending aorta. The third patient was a 23-year-old woman with a previous diagnosis of congenital aortic stenosis. Her characteristic elfic facies induced us to suspect the syndrome of Williams-Beuren; transthoracic and transesophageal echocardiographic examination showed an hour-glass supravalvular aortic stenosis with a peak gradient of 60 mmHg. Magnetic resonance imaging showed hypoplasia of the descending aorta and the iliac arteries. Since she was asymptomatic and presented only with a moderate gradient, the patient was not referred to surgical therapy. In this manuscript we present the three cases and review the histopathological, clinical, genetic, diagnostic and therapeutic aspects of this disease and its natural history.

[Heart rupture in acute myocardial infarct: the advantages of using M-2D color Doppler echocardiography in a coronary intensive therapy unit]. / Rottura di cuore nell'infarto acuto del miocardio: vantaggi dell'uso dell'ecocardiografia M-2D color Doppler in unità di terapia intensiva coronarica.

BACKGROUND: Free-wall rupture of the heart is the second most common cause of death in acute myocardial infarction (AMI), following pump failure. Acute rupture is more common and rapidly fatal, while subacute rupture, which accounts for about 30% of total cases of mortality in AMI, can be diagnosed early by clinical signs with the support of echocardiography in coronary intensive care units. METHODS: From March 1996 to December 1997, 293 patients diagnosed with acute myocardial infarction were admitted to the coronary intensive care unit of our hospital. Of these patients, 71 (23.8%) were treated with thrombolysis within 6 hours of onset of symptoms. All patients were observed daily with M-2D color Doppler echocardiography and in the event of renewed chest pain, electrocardiogram changes, abrupt hypotension, syncope or clinical signs of low output syndrome. RESULTS: We observed 11 cases (3.8%) of free-wall rupture of the heart in acute myocardial infarction with echocardiography, 6 females and 5 males, with a mean age of 74.2 +/- 7.8 years (min. 56-max 84), none of whom had prior AMI. Six of them received thrombolytic therapy, six were hypertensive (54.5%) and three were diabetics (27.2%). Surgical repair was performed in two patients with subacute rupture, but one died a few days later. The echocardiography data at bedside for diagnosis of cardiac rupture were confirmed in 5 patients with autopsy and intraoperatively in two of them. CONCLUSIONS: Routine use of echocardiography in coronary intensive care units allows prompt diagnosis of cardiac rupture in acute myocardial infarction, and in the event of subacute rupture it can accelerate surgical decision-making.

[Rotational atherectomy and PTCA in complex coronary lesions (B2 and C): the immediate and long-term results]. / Aterectomia rotazionale e PTCA in lesioni coronariche complesse (B2 e C): risultati immediati e a distanza.

BACKGROUND: Percutaneous transluminal coronary angioplasty (PTCA) in complex coronary lesions (type B2 and C of the modified AHA/ACC classification) presents a lower primary success rate and higher risk of dissection than type A and B1 lesions. An alternative approach to this lesions is coronary rotational ablation (Rotablator, Heart Technology) with complementary PTCA using low inflation pressures ("facilitated angioplasty"). MATERIALS AND METHODS: Twenty-six type B2 and C lesions in 24 patients (pts) (8 female, 16 male, age 37-80 years) were treated with coronary rotational ablation and complementary PTCA between January 1993 and December 1994 (4.7% of all interventional coronary procedures performed in this period in our laboratory). Eleven pts had stable effort angina and 13 pts had unstable, class IB, IIB, and IIC, angina. The treated vessel was the LAD in 15 cases, CX in 5, RCA in 5, and an intermediate branch in one case. Coronary rotational ablation was proposed because of the presence of two or more risk factors for uneffective or complicated PTCA: eccentricity, calcified lesions, bifurcation stenosis, lesion length > 10 mm, severe stenosis (90-99%), ostial location and bend location (45-60 degrees). No lesion showed coronary thrombus, considered as absolute contraindication to coronary rotational ablation. We used small burrs (burr/artery ratio < 0.75), and complementary PTCA was performed using low inflation pressure (< 8 atm) and long balloons for long lesions (> 10 mm) in order to minimize the risk of dissection. RESULTS: Coronary rotational ablation was successfully performed in all but two cases (24/26; 92.3%), with a reduction of the stenosis from 88 +/- 9% to 45 +/- 10% (range 30-60%). In two pts (7.7%) the procedure was complicated by acute occlusion: both pts underwent effective salvage PTCA with 30% residual stenosis. Small type A and B dissections occurred in 4/26 cases (15.4%). All but one lesions complicated by acute occlusion or dissection following coronary rotational ablation were not or only slightly calcified. Complementary PTCA was performed in all but two pts who already presented 30% residual stenosis after rotational ablation. A further reduction of stenosis to 20 +/- 9% (range 5-30%) was achieved. After complementary PTCA four pts (15.4%) developed type A and B dissections; in one of these a Palmaz-Schatz stent was implanted, whereas the remaining three pts presented a residual stenosis below 30% and no further procedures were undertaken. Overall success rate of rotational atherectomy plus salvage or complementary PTCA or stenting was 100%, and no major complications (Q-wave myocardial infarction, emergency bypass surgery or death) occurred. Three pts showed delayed coronary run-off (slow reflow) after rotational ablation, and two of these released a small amount of cardiac specific enzymes (CK MB) without ECG changes and wall motion alteration on echocardiographic examination. Clinical restenosis, defined as recurrent angina and/or positive exercise stress test, developed in 45.8% (11 pts); in all these pts restenosis was angiographically evidenced (75-99%). CONCLUSIONS: Our experience suggests that coronary rotational ablation along with complementary PTCA using low inflation pressure and long balloons is safe and effective in type B2 and C lesions if calcifications are present; however, restenosis rate remains high.

[Regression of pulmonary hypertension in mitral stenosis: an echo-hemodynamic study in patients who underwent mitral balloon valvuloplasty]. / Regressione dell'ipertensione polmonare nella stenosi mitralica: studio eco-emodinamico in pazienti sottoposti a valvuloplastica mitralica con palloncino.

BACKGROUND: Reversibility of pulmonary hypertension in patients affected by mitral stenosis is still under question. METHODS: We selected 80 patients (mean age 48 +/- 14) who underwent successful percutaneous mitral valvuloplasty (PMV) for hemodynamic significant mitral stenosis (area < 1.5 cm2) with pulmonary hypertension (mean artery pulmonary pressure--PPM > 25 mm Hg), producing significant increase in mitral valve area (area before PMV = 0.99 +/- 0.23 cm2 vs 2.08 +/- 0.32 cm2 after PMV--p < 0.001) without hemodynamic complications (mitral insufficiency and/or interatrial shunt). Cardiac index, pulmonary arterial pressures, and pulmonary arteriolar resistances were invasively evaluated before and immediately after valvuloplasty. Systolic pulmonary pressure was indirectly monitored by Doppler method in a period from 1 to 3 months after percutaneous mitral valvuloplasty. RESULTS: In general (70 pts.) there was an immediate significant reduction of pulmonary pressure after percutaneous mitral valvuloplasty (mean pulmonary pressure before PMV was 33.9 +/- 7.9 mm Hg vs 26.8 +/- 9.5 mm Hg after PMV, p < 0.01; systolic pulmonary pressure before PMV was 51.5 +/- 10.9 mm Hg vs 43.15 +/- 13.5 mm Hg after PMV--p < 0.01). A small subgroup of 10 pts., older in age (mean 59 +/- 15), manifested no reduction of pulmonary pressure immediately after procedure (mean pulmonary pressure before PMV = 35.2 +/- 8.37 mm Hg vs 36.5 +/- 6 mm Hg after PMV, p: ns; systolic pulmonary pressure before PMV = 58.2 +/- 10.6 mm Hg vs 59.2 +/- 9.6 mm Hg. after PMV, p: ns) and 4 of them (mean age 65 +/- 15) persisting pulmonary hypertension at 1-3 months follow-up (systolic pulmonary pressure before PMV = 58.75 +/- 14 mm Hg, immediately after PMV = 57.8 +/- 12.5 mm Hg, and 1-3 months after PMV = 62.5 +/- 9 mm Hg--p: ns). CONCLUSIONS: Neither severe pulmonary hypertension, nor pulmonary arteriolar resistances but only age seems to be a predictive factor of persisting pulmonary hypertension after percutaneous mitral valvuloplasty in mitral stenosis.