Estimulación bicameral en una paciente con miocardiopatía hipertrófica obstructiva refractaria y mala tolerancia a la ultrafiltración extracorpórea / Dual-chamber pacing in a patient with refractory obstructive hypertrophic cardiomyopathy and intolerance to extracorporeal ultrafiltration

El tratamiento óptimo de los pacientes con miocardiopatía hipertrófica obstructiva refractaria al tratamiento médico convencional es motivo de controversia. Algunos autores han demostrado la eficacia, en cuanto a mejoría sintomática y reducción del gradiente dinámico, de la estimulación secuencial auriculoventricular en este tipo de pacientes. Se presenta el caso de una paciente trasplantada renal, con miocardiopatía hipertrófica obstructiva con gradiente subaórtico elevado, que ingresó en la unidad coronaria por insuficiencia cardíaca refractaria y deterioro de la función renal; precisó ultrafiltración extracorpórea que no toleraba de manera reiterada por episodios graves de bajo gasto. Se comentan los aspectos terapéuticos y se destaca la buena respuesta a la estimulación con marcapasos bicameral. La utilidad de la estimulación secuencial en pacientes con miocardiopatía hipertrófica obstructiva sometidos a ultrafiltración por presentar síntomas congestivos refractarios al tratamiento médico convencional no ha sido previamente comunicada (AU)

Anomalous coronary artery origin associated with bicuspid aortic valve in a patient with rheumatic mitral stenosis: a case report.

A rare case of an anomalous left coronary artery arising from the right sinus of Valsalva associated with bicuspid aortic valve is presented. This case is unique because these congenital anomalies were associated with rheumatic mitral stenosis. This anomalous coronary origin was found at catheterization before balloon mitral valvuloplasty. The clinical significance of this finding is discussed.

[Obstructive myocardiopathic hypertrophy and sequential atrioventricular stimulation. Immediate results and long term follow-up. Seven years experience]. / Miocardiopatía hipertrófica obstructiva y estimulación secuencial auriculoventricular. Resultados agudos y seguimiento a largo plazo. Siete años de experiencia.

BACKGROUND: Sequential pacing as a primary treatment for patients with severe hypertrophic obstructive cardiomyopathy is still under controversy. In this study we sought to evaluate the effects of pacing on left ventricular systolic and diastolic function, its repercussion on pathophysiology and the possible changes in clinical evolution and functional class in patients who didn't respond to medical therapy. PATIENTS AND METHODS: Thirty-four patients with severe hypertrophic obstructive cardiomyopathy in functional class III-IV underwent evaluation. The mean age was 64 +/- 11 years. Sixty-two per cent were over 65. The study protocol included Holter monitoring, baseline echo-Doppler, during pacing, at 6 months and at the end of follow-up, and a complete catheterization and coronary angiography. Mean follow-up time was 36 +/- 20 month (range 6-74). In four patients sequential pacing was ineffective and the protocol had to be discontinued. Thirty patients received a permanent dual-chamber pacemaker and systolic and diastolic function were evaluated by hemodynamic, angiographic and eco-Doppler studies. RESULTS: Long-term stimulation reduced the subaortic gradient from 95 +/- 38 to 39 +/- 28 mmHg (p < 0.001), left ventricular systolic pressure from 207 +/- 38 to 164 +/- 30 mmHg (p < 0.001) and left ventricular end-diastolic pressure from 23 +/- 7 to 14 +/- 5 mmHg (p < 0.001), pulmonary capillary wedge pressure from 19 +/- 7 to 14 +/- 5 mmHg (p < 0.001), ejection fraction from 79 +/- 6 to 71 +/- 6% (p < 0.001) and mitral insufficiency. Diastolic function improved with an increase in early filling from 39 +/- 11 to 52 +/- 10% (p < 0.001) and E/A relation from 0.93 +/- 0.4 to 1.6 +/- 0.8 (p < 0.001), and a reduction in late filling from the left ventricle from 36 +/- 10% to 24 +/- 10% (p < 0.001). The speed of the E wave increased from 0.83 +/- 0.3 to 0.92 +/- 0.3 (p < 0.001) and we observed a reduction of the A wave from 93 +/- 0.3 to 0.66 +/- 0.2 (p < 0.001), pressure-half time from 95 +/- 34 to 66 +/- 19 ms (p < 0.001) and we observed deceleration time from 320 +/- 90 to 221 +/- 57 (p < 0.01). All patients have improved their functional class, allowing a reduction in pharmacological therapy. Two exitus of cardiological origin occurred. CONCLUSIONS: Sequential AV pacing in selected patients with severe hypertrophic obstructive cardiomyopathy can reduce subaortic gradient, pulmonary capillary wedge, left ventricular filling pressures and the severity of mitral regurgitation, and improve the markedly altered diastolic function. The significant symptomatic improvement allows a reduction in medical treatment. Thus, it represents a viable alternative to open-heart surgery, particularly in elderly patients, with lower morbidity and mortality rates.

[Impact of electric cardiac stimulation on ventricular function and the natural history of patients with myocardiopathy]. / Impacto de la estimulación cardíaca eléctrica sobre la función ventricular y la historia natural de los pacientes con miocardiopatía.

There has recently been an increasing interest in beneficial effects of cardiac pacing in patients with myocardial diseases, especially in Obstructive Hypertrophic and Dilated Cardiomyopathy. The experience with dual-chamber pacing for obstructive hypertrophic cardiomyopathy is now important. DDD pacing for sinus rhythm patients and VVI pacing in patients with atrial fibrillation have shown considerable symptomatic improvement, with a significant decrease of angina, dyspnea, presyncope and frank syncope. It has been suggested that DDD pacing may prevent sudden death and improve survival rates in these patients, but this has not yet been established. The experience with DDD pacing in dilated cardiomyopathy is more limited, but in specially chosen patients, DDD pacing with short AV delay has shown symptomatic improvement and a decrease in the need for further hospitalization due to worsening of heart failure. There is no current evidence of higher survival rates with this treatment, but DDD pacing may be used in patients with end-stage dilated and isquemic cardiomyopathy who are waiting for a heart transplantation.

Different hemodynamic responses between acute and chronic infusion of iloprost (prostacyclin-stable analogue) in severe pulmonary hypertension.

We report the hemodynamic and clinical effects of acute and chronic administration of iloprost in two patients with severe pulmonary hypertension caused by toxic oil syndrome. We tested the acute effect of progressive increments of iloprost, followed by long-term infusion of the drug during 14 days. The acute response produced an increase in cardiac output and moderate reduction in pulmonary vascular resistance, with no change in pulmonary artery pressure. Nevertheless, a maintained reduction in pulmonary artery pressure and resistance, as well as clinical improvement, was observed after chronic infusion. We conclude that (1) the acute effect of iloprost does not necessarily predict long-term hemodynamic response, and (2) iloprost given in long-term infusion seems to have been an efficacious and safe drug in our two patients, and it opens a new line of treatment.