Repair of Atrial Septal Defect With Eisenmenger Syndrome After Long-Term Sildenafil Therapy

doi:10.1016/j.athoracsur.2009.10.027

The Annals of Thoracic Surgery

Volume 89, Issue 5, May 2010, Pages 1629-1630

https://doi.org/10.1016/j.athoracsur.2009.10.027 Get rights and content

We report a woman with atrial septal defect and severe pulmonary hypertension with 25.0 Wood unit · m² of indexed total pulmonary vascular resistance. She underwent successful corrective repair of atrial septal defect after 2 years of treatment with sildenafil, and has been monitored for 4 years after repair. This case supports a “treat and repair” approach using advanced pulmonary vasodilator therapy in selected patients with inoperable severe pulmonary hypertension associated with atrial septal defect.

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Several cases compatible with the treat and repair approach for patients with ASD and severe pulmonary hypertension have been reported [2, 3, 4]. Frost and colleagues [2] described a 29-year-old woman with near-systemic pulmonary hypertension who underwent defect closure after a drop in pulmonary pressure with continuous intravenous prostacyclin. Schwerzmann and colleagues [3] described a 38-year-old woman (PVR of 8.8 WU, reduced to 4.2 WU with adenosine), who showed significant symptomatic and

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Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy
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A.E. Frost et al.
Reversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol
J Heart Lung Transplant
(2005)
M. Schwerzmann et al.
Atrial septal defect closure in a patient with ‘irreversible’ pulmonary hypertensive arteriopathy
Int J Cardiol
(2006)

There are more references available in the full text version of this article.

Cited by (27)

Transcatheter Closure of Atrial Septal Defect Associated With Pulmonary Artery Hypertension using Fenestrated Devices
2021, American Journal of Cardiology
Citation Excerpt :
In addition to the acute pulmonary vasoreactivity test, the balloon occlusion test can be helpful for evaluating “operability”.25 The “treat-and-repair” strategy has been applied in clinical practice with favorable short to mid-term results.26 –28 Long-term data of application of treat-and-repair strategy are very few.
In patients with pulmonary artery hypertension (PAH) associated with atrial septal defect (ASD), closure of ASD may carry significant risks. We aimed to evaluate the safety and efficacy of transcatheter closure of ASD in selected patients with PAH using a fenestrated device followed by pulmonary vasodilator therapy. During the 14.8-year period, 51 ASD patients (10 males, age 46 ± 18 years) with a mean pulmonary artery pressure (PAP) ≥ 35 mm Hg and/or systolic PAP ≥ 60 mm Hg, underwent closure with a fenestrated device. Of them, mean Qp/Qs ratio, systolic PAP and mean PAP were 2.6 ± 1.2, 73 ± 14 mm Hg, and 44 ± 8 mm Hg, respectively. A total of 35 patients received pulmonary vasodilator therapy. The New York Heart Association (NYHA) functional class improved at 3 to 6 months follow-up. (p < 0.001) Nineteen patients underwent repeated catheterization. A comparison of the hemodynamic parameters between baseline and repeated catheterization revealed significant decreases in both systolic and mean PAP (77 ± 11 vs 55 ± 14 mm Hg, p < 0.0001 & 48 ± 7 vs 37 ± 8 mm Hg, p = 0.001, respectively), pulmonary vascular resistance (PVR) (5.1 ± 2.3 vs 4.0 ± 1.7 WU, p = 0.011) and PVRi (7.7 ± 3.3 vs 6.2 ± 2.4 WU*m2, p = 0.024). After a follow-up period of 84 ± 45 months, 6 mortalities were noted in which 2 were due to cardiac causes. In conclusion, catheter closure of ASD in patients with PAH using a fenestrated device followed by vasodilator therapy is safe and effective.
Early pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6 months of age
2017, International Journal of Cardiology
Citation Excerpt :
If PVR/SVR is 0.2–0.3, VSD closure may be undertaken with high peri- and postoperative risk of morbidity and mortality [14]. In patients with elevated PVR with PVR/SVR > 0.3, an individual treatment options such as unidirectional valved patch closure of ventricular septal defect [15] or preoperative pulmonary vasodilator therapy [16,17] must be considered. However, the prognosis concerning development of PAH after VSD closure, particularly of patients with preoperatively borderline pulmonary vascular hemodynamics is unclear.
Pulmonary arterial hypertension (PAH) is virtually absent after closure of ventricular septal defect (VSD) in the first six months of life. However the prevalence of PAH in patients, who underwent VSD closure later, is not clear. The aim of this study was to analyse the prevalence of PAH after a successful VSD closure after the age of 6 months and whether there are risk factors for developing PAH.
Echocardiographic and right heart catheter data of patients with VSD or complete atrioventricular septal defect, who underwent VSD closure after the age of 6 months in our institution between 01/2005 and 06/2014, were retrospectively analyzed. PAH was defined as mean pulmonary arterial pressure (mPAP) of ≥ 25 mm Hg or tricuspid regurgitation jet velocity of ≥ 3.5 m/s.
In 228 patients (median age at shunt closure 4.0 years, range 0.5–69) and 174 complete follow-up data (median follow-up 3.7 years, range 0.5–39.4), 9 patients needed pulmonary vasodilator therapy after shunt closure, 4 of them temporarily for up to 79 months. Three patients are still on vasodilator treatment 1, 2.6 and 6 years after surgery, other two were lost to follow-up. Another 6 patients with preoperatively borderline hemodynamics due to elevated mPAP and pulmonary vascular resistance, recovered well without signs of postoperative PAH.
With the current practice for safe late VSD closure, PAH is very rare at least in the first years of follow-up. In most patients with perioperative PAH, this condition appears to be transient and shows good response on pulmonary vasodilator treatment.
What radiologists need to know about the pulmonary-systemic flow ratio (Qp/Qs): What it is, how to calculate it, and what it is for
2015, Radiologia
La resonancia magnética cardiaca aporta abundante información morfológica y funcional al estudio de las cardiopatías congénitas. Entre los datos funcionales se encuentran el gasto pulmonar y el gasto sistémico; el cociente entre ambos es el cociente Qp/Qs. Después del nacimiento, en condiciones normales el gasto pulmonar y el sistémico son prácticamente iguales, y el cociente Qp/Qs = 1. En los pacientes con cortocircuitos entre la circulación sistémica y la pulmonar este cociente se altera. Dependiendo de la localización del cortocircuito (intra o extracardiaco), y de las alteraciones estructurales o posquirúrgicas asociadas, la interpretación de los hallazgos es diferente. Revisamos el concepto de Qp/Qs, los métodos para calcularlo con especial énfasis en la RM, y el significado de los resultados obtenidos, haciendo hincapié en la relevancia de estos datos dependiendo de la patología de base y los procedimientos terapéuticos que se hayan realizado al paciente.
Cardiac magnetic resonance imaging (cMRI) provides abundant morphological and functional information in the study of congenital heart disease. The functional information includes pulmonary output and systemic output; the ratio between these two (Qp/Qs) is the shunt fraction. After birth, in normal conditions the pulmonary output is practically identical to the systemic output, so Qp/Qs = 1. In patients with «shunts» between the systemic and pulmonary circulations, the ratio changes, and the interpretation of these findings varies in function of the location of the shunt (intracardiac or extracardiac) and of the associated structural or postsurgical changes. We review the concept of Qp/Qs; the methods to calculate it, with special emphasis on cMRI; and the meaning of the results obtained. We place special emphasis on the relevance of these findings depending on the underlying disease and the treatment the patient has undergone.
CASE 10 2014 Eisenmenger syndrome: Close the hole?
2014, Journal of Cardiothoracic and Vascular Anesthesia
Citation Excerpt :
Nonetheless, Kim et al later demonstrated successful ASD closure in a 41-year-old female with a large ASD and ES (PVR 25 Wood units) following 2 years of sildenafil treatment with symptomatic improvement and resolution of cyanosis. She was weaned off all pulmonary vasodilators 3 years post-repair, and at her 6-year follow-up, she was symptom free.15 Clinical arguments in favor of repair of the defect include the significant risk these patients have for cerebrovascular events, exercise limitations, and peripheral organ dysfunction secondary to systemic arterial desaturation.13
Atrial septal defects
2014, The Lancet
Citation Excerpt :
American and European practice guidelines86,87 state that an atrial septal defect can be closed if the pulmonary vascular resistance is lower than two-thirds of the systemic vascular resistance (at baseline or after pulmonary vasodilator acute challenge or targeted pretreatment course) and there is evidence of a pulmonary-to-systemic flow ratio greater than 1·5 (class IIB, level of evidence C). A calculated pulmonary vascular resistance greater than 8 Woods units generally precludes closure, as does a resting interatrial right-to-left shunt such as Eisenmenger syndrome.88 Closure can also be contraindicated in other conditions in which the defect serves as a decompressing route for blood flow (pop-off valve), including severe obstructive or restrictive right or left heart lesions.87,89
Atrial septal defects are the third most common type of congenital heart disease. Included in this group of malformations are several types of atrial communications that allow shunting of blood between the systemic and the pulmonary circulations. Most children with isolated atrial septal defects are free of symptoms, but the rates of exercise intolerance, atrial tachyarrhythmias, right ventricular dysfunction, and pulmonary hypertension increase with advancing age and life expectancy is reduced in adults with untreated defects. The risk of development of pulmonary vascular disease, a potentially lethal complication, is higher in female patients and in older adults with untreated defects. Surgical closure is safe and effective and when done before age 25 years is associated with normal life expectancy. Transcatheter closure offers a less invasive alternative for patients with a secundum defect who fulfil anatomical and size criteria. In this Seminar we review the causes, anatomy, pathophysiology, treatment, and outcomes of atrial septal defects in children and adult patients in whom this defect is the primary cardiac anomaly.
Atrial septal defect closure
2013, Cardiology Clinics

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Case reportRepair of Atrial Septal Defect With Eisenmenger Syndrome After Long-Term Sildenafil Therapy

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Int J Cardiol

J Heart Lung Transplant

Int J Cardiol

Case report
Repair of Atrial Septal Defect With Eisenmenger Syndrome After Long-Term Sildenafil Therapy