sildenafil-citrate and Mitral-Valve-Stenosis

The patency of foramen ovale (FO) in fetal circulation is very important, and premature closure of FO could be associated with several pathological conditions.. We report a patient in whom premature closure of FO in fetal life was associated with late clinical onset of mitral valve stenosis and subsequent development of irreversible pulmonary hypertension (PH).. The patient showed persistent PH after birth, which completely regressed at the age of 8 months. However, the patient developed heart failure due to mitral valve lesions (hammock valve) at the age of 11 months and underwent artificial valve replacement. The patient subsequently developed severe PH, which was refractory to anti-PH therapy with sildenafil and bosentan in addition to home oxygen.. This case illustrates that mitral stenosis can be overlooked during early neonatal life, and thus emphasizes the need for close follow-up for potential existence of mitral stenosis and later clinical manifestation in patients with premature FO closure even when initial careful examination of the mitral valves does not indicate any abnormalities. In addition, premature closure of FO could cause pulmonary vascular disease, which may lead to later development of irreversible PH.

Topics: Antihypertensive Agents; Bosentan; Cardiac Catheterization; Fetal Diseases; Foramen Ovale; Heart Failure; Heart Valve Prosthesis Implantation; Humans; Hypertension, Pulmonary; Infant, Newborn; Male; Mitral Valve Stenosis; Piperazines; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Treatment Failure; Ultrasonography, Doppler, Pulsed; Ultrasonography, Prenatal; Vasodilator Agents

Pulmonary hypertension is a serious disorder, difficult to treat especially in the severe forms. The treatment consists mainly of calcium channel blockers, anti-coagulation, intravenous epoprostenol, inhaled nitric oxide and recent agents as bosentan and sildenafil. Sildenafil, a phosphodiesterase 5 specific inhibitor, has been largely evaluated in primary pulmonary hypertension, and in some cases of secondary pulmonary hypertension including parenchymal and thromboembolic diseases; it has not yet been evaluated in severe pulmonary hypertension with elevated pre-capillary resistance in operated mitral stenosis. We report the cases of two patients operated from mitral valve replacement for severe mitral stenosis with elevated pre-capillary resistance, where oral sildenafil, introduced empirically immediately after the surgical procedure at the dose of 50 mg/d, permitted a significant decrease in pulmonary pressures and resistances, allowing a rapid withdrawal of nitric oxide and reducing therefore hospitalization time in the intensive care unit. We think that this simple treatment, with or without association to nitric oxide, should be generalized to persistent pulmonary hypertension following cardiac surgery.

Topics: Capillaries; Female; Humans; Hypertension, Pulmonary; Middle Aged; Mitral Valve Stenosis; Phosphodiesterase Inhibitors; Piperazines; Postoperative Care; Purines; Severity of Illness Index; Sildenafil Citrate; Sulfones

Pulmonary hypertension (PHT) is a rare entity that is difficult to treat. Prognosis is poor. Sildenafil, a selective inhibitor of type 5 phosphodiesterase, has been proposed among the many treatments available for primary and secondary pulmonary hypertension. We report our experience with an infant with pulmonary hypertension due to congenital mitral stenosis and persistent ductus arteriosus, who developed congestive cardiac failure with persistent PHT despite surgical correction. Conventional treatment was unsuccessful and the patient was treated with sildenafil. The clinical course was satisfactory, allowing extubation and withdrawal of vasoactive drugs; pulmonary and left atrial pressure decreased and the patient was discharged. She is currently being treated on an outpatient basis with oral sildenafil and shows satisfactory hemodynamic status. We review alternatives to conventional treatments for pulmonary hypertension with special reference to pediatrics.

Topics: Ductus Arteriosus, Patent; Humans; Hypertension, Pulmonary; Infant; Mitral Valve Stenosis; Phosphodiesterase Inhibitors; Piperazines; Purines; Sildenafil Citrate; Sulfones; Vasodilator Agents

Topics: Administration, Inhalation; Drug Synergism; Heart Valve Prosthesis Implantation; Humans; Hypertension, Pulmonary; Infant; Male; Mitral Valve Stenosis; Nitric Oxide; Piperazines; Postoperative Complications; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome