title: Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment.
abstract: The fundamental role of pulmonary vascular resistance in the Fontan circulation is obvious. Medications decreasing this resistance may have an impact on the fate of this population. Hence, we assessed noninvasively the effect of oral sildenafil on the ventriculo-arterial coupling in patients with Fontan circulation. In a single-center, prospective case series study, 23 patients with fenestrated extracardiac total cavopulmonary connection age 12-31 years were enrolled in this study. Clinical characteristics and echocardiographic examination were performed before and after a 1 week course of sildenafil at 0.5 mg/kg every 8 h. Sildenafil had no effect on heart rate and blood pressure. However, oxygen saturation was significantly increased with sildenafil (87.6 ± 4.3 vs. 90.1 ± 3.6; P < 0.0001). The calculated noninvasive ventricular end-systolic elastance (Ees) was greater after sildenafil compared with the pre-sildenafil values (1.59 ± 0.17 vs. 1.72 ± 0.27 mm Hg/ml; P = 0.001). Moreover, significant decreases in arterial elastance (Ea) (1.62 ± 0.53 vs. 1.36 ± 0.43 mm Hg/ml; P < 0.0001), ventricular end-diastolic elastance (Eed) (0.05 ± 0.021 vs. 0.04 ± 0.013; P = 0.002), and, finally, ventriculo-arterial coupling index (0.99 ± 0.26 vs. 0.76 ± 0.15; P < 0.0001) were found after sildenafil administration. The intolerable side effects that led to stopping the sildenafil occurred only in one (4 %) patient. Sildenafil has increased ventricular systolic elastance and improved ventriculo-arterial coupling in patients palliated with Fontan circulation. Short-term sildenafil was well tolerated in most of the patients with only minor side effects.
authors.affiliation: Department of Pediatric Cardiology, Children's Medical Center, Tehran University of Medical Sciences, 14194 Tehran, Iran. rzshabanian@sina.tums.ac.ir
authors.forename: Reza
authors.initials: R
authors.lastname: Shabanian
authors.forename: Leila
authors.initials: L
authors.lastname: Shahbaznejad
authors.forename: Anahita
authors.initials: A
authors.lastname: Razaghian
authors.forename: Abdolrazagh
authors.initials: A
authors.lastname: Kiani
authors.forename: Mitra
authors.initials: M
authors.lastname: Rahimzadeh
authors.forename: Soroush
authors.initials: S
authors.lastname: Seifirad
authors.forename: Armen
authors.initials: A
authors.lastname: Kocharian
authors.forename: Jami Shakibi
authors.initials: JS
authors.lastname: Gilani
authors.forename: Mohammad Ali
authors.initials: MA
authors.lastname: Navabi
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004452:Echocardiography / Q000379:methods*; D005260:Female; D018610:Heart Bypass, Right / Q000379:methods*; D006801:Humans; D008297:Male; D010879:Piperazines / Q000008:administration & dosage* / Q000009:adverse effects; D011446:Prospective Studies; D011687:Purines / Q000008:administration & dosage / Q000009:adverse effects; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage* / Q000009:adverse effects; D014655:Vascular Resistance / Q000187:drug effects*; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects; D055815:Young Adult
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate