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.
Atrial septal defect (ASD) accounts for approximately 8% of congenital heart disease. Because the symptoms are generally subtle, ASD frequently remains undetected in childhood. Adults with unrepaired ASD may have pulmonary artery hypertension (PAH), heart failure, arrhythmia and even death. – Since PAH is common in adults with an open ASD and pulmonary vascular disease may progress with time, closure of ASD should be performed within the appropriate time period. If patients have Eisenmenger syndrome or severe pulmonary vascular obstructive disease, closure of ASD is contraindicated.
Partial closure of ASD has been advocated in patients with PAH and can be achieved by creating a small fenestration in the surgical patch. This enables gradual reduction in left-to-right shunt and prevents drastic changes in hemodynamics in those patients. We developed a strategy to close ASD with moderate-to-severe PAH by using fenestrated devices followed by targeted therapy. –
Methods
In a 14.8-year period between July 2002 and March 2017, 55 patients diagnosed as having unrepaired secundum type ASD and moderate-to-severe PAH, defined as a mean (PAP) ≥ 35 mm Hg and/or systolic PAP ≥ 60 mm Hg , and PVR < 12 Wood Unit (WU), underwent attempted transcatheter closure with a fenestrated device. Clinical evaluation, ECG and echocardiography were performed before the procedure. Four patients were excluded from this procedure, because of very high PVR or intolerance to balloon occlusion test. This study has been approved by the ethical committee of this institution. There were 51 patients, 41 women (80%) and 10 men, undergoing transcatheter closure using a fenestrated device. Their ages ranged from 16 to 75 years (46 ± 18 years, median 43 years). Before intervention, 3 patients had been treated with pulmonary vasodilators in other hospitals. One had undergone an unsuccessful surgical closure 8 years in a foreign country before the intervention. Twelve patients (24 %) had atrial arrhythmia: atrial fibrillation in 8 and atrial flutter in 4. Of them, 1 with atrial fibrillation underwent catheter ablation and 2 patients with atrial flutter underwent cardioversion prior to the procedure.
Informed consent was obtained from each patient. After local anesthesia was administered, hemodynamic studies were performed and PVR was obtained. An acute vasodilation test was performed in selected patients with PVR > 6 WU*m2 or PVR/SVR > 0.3. , Following general anesthesia, transesophageal echocardiography (TEE) was used to monitor the procedure.
The Amplatzer septal occluders (AGA Medical Corporation, Plymouth, MN, USA) were used in all patients. The device size selected is usually equal to or up to 2 mm larger than the balloon sizing diameter. Since 2006, the majority of patients did not undergo balloon sizing. Without balloon sizing, the device size selected was generally 4–7 mm or 20%–25% larger than maximal diameter of ASD on TEE images. A fenestration approximately one-fourth of device diameter was manually created by removing part of the patch. , In many patients, an additional fenestration of approximately 4–5 mm in diameter was made to ensure adequate flow across the fenestration after deployment ( Figure 2 ). After closure, Aspirin 200 mg was administrated for at least 6 months except to those who were taking warfarin. Clinical evaluation, electrocadiogram and transthoracic echocardiography were performed at 1 day, 1 week, 1 month, 3 months and 6 months after the procedure, then every 6 months afterwards. The NYHA functional class was evaluated before intervention and at 3- or 6-months follow-up in each patient. Pulmonary vasodilators were given in patients with echocardiographic estimated tricuspid valve regurgitation gradient > 46 mm Hg or systolic PAP > 50 mm Hg during the 1- or 3-months follow-up.
Data are presented as mean ± standard deviation (STD). Student’s t test or 1 way analysis of variance (ANOVA) were used for a numerical data comparison. Paired t test was used to compare the hemodynamic parameters at baseline and during follow-up. The Chi-square test and Fisher’s exact test were used for a categorical data comparison. Logistic regression was used for multivariate risk factor analysis of persistent PAH.
Results
The demographic data and follow-up data of the 51 patients are summarized in Table 1 . A fenestrated occluder was successfully deployed in all 51 patients. Shunt flows across fenestrations were observed on TEE images for all patients immediately after device implantation. Fourty nine patients were discharged within 2 days after the procedure. No patients developed pulmonary hypertension crisis during and after the procedure.
| Baseline | Follow-up | p value | |
|---|---|---|---|
| Age (years, mean ± STD) | 46±18 | ||
| Women | 41 (80%) | ||
| Function class | |||
| I | 0 | 11 (22%) | <0.001 |
| II | 27 (53%) | 33 (65%) | |
| III | 19 (37%) | 7 (14%) | |
| IV | 5 (10%) | 0 | |
| Pro-brain natriuretic | 1722 ± 3506 | 412 ± 705 | 0.044 |
| peptide (pg/mL) | 24 | 16 | |
| Atrial flutter/fibrillation (%) | 12 (24%) | 8 (16%) | 0.318 |
| Estimated PAP by echocardiography (mm Hg) | 76 ± 21 | 48 ± 24 | <0.001 |
| Cardiac catheterization | |||
| systolic PAP (mm Hg) | 73 ± 14(50∼105) | 55 ± 14 * (33∼80) | <0.001 * |
| mean PAP (mm Hg) | 44 ± 8 (28∼61) | 37 ± 8 * (24∼50) | 0.001 * |
| Qp/Qs | 2.6 ± 1.2(1∼5.8) | ||
| estimated PVR (WU) | 4.9 ± 2.9 (1.2∼13.7) | 4.0 ± 1.7 * (1.8∼10.5) | 0.011 * |
| estimated PVRi (WU * m2) | 7.3 ± 4.3(1.9∼19.4) | 6.2 ± 2.4 * (3∼14.8) | 0.024 * |
| ASD | |||
| Maximal diameter on echocardiography (mm) | 27 ± 6 | ||
| Device size (mm) | 33 ± 6(22–40) | ||
| Follow-up period (months) | 84 ± 45 |
⁎ for 19 patients received follow-up cardiac catheterization paired-t test
In the 3 patients who received pretreatment, pulmonary vasodilator therapy was continued immediately after the procedure. In 32 patients, Sildenafil 20 mg twice or 3 times daily was given as first-line therapy after evaluation with Doppler echocardiography in the 1- or 3-month follow-up. A total of 35 patients received vasodilator therapy. Bosentan was added based on echocardiographic estimation of systolic PAP in 5 patients for more efficient control of PAH during follow-up. Forty-nine patients received at least 6-month of follow-up. Two were lost to follow-up after the third month visit. A second cardiac catheterization was performed to evaluate the hemodynamics in 19 patients at 4.5–92 (mean 40 ± 35) months after the procedure. The mean Qp/Qs was 1.4 ± 0.4 (1–2.6). A comparison of hemodynamic parameters obtained at baseline with those obtained at repeated catheterization were shown in Figure 1 . The fenestrations were still patent in 15 of 19 (79%) patients at the time of second catheterization. Transcatheter closure of the fenestration was performed in the three patients who had a significant shunt across the fenestration with Qp/Qs ratio > 2. Two patients underwent balloon dilation of the fenestration and 7 patients underwent concomitant stent implantation (Express SD stent, 7 mm diameter by 19 mm long, Boston Scientific) at the fenestration to maintain interatrial communication, because PAP remained high (mean PAP > 25 mm Hg) ( Figure 2 ). After a mean follow-up period of 84 ± 45 months, there were 6 deaths: 4 were non-cardiac death and 2 mortalities were likely cardiac causes. ( Figure 3 ) Of the 2 patients dying of cardiac causes, 1 received vasodilator treatment and the other had poor compliance to vasodilator treatment ( Table 2 ). Two patients had uneventful deliveries of babies after the second catheterization. In the most recent follow-up, left-to-right shunt across the fenestrations could be observed on precordial echocardiography in 21 patients. No 1 had desaturation during follow up. Significant improvements in NYHA functional class was observed during follow-up ( Table 1 ).
| No | Age | Sex | baseline PAP (mm Hg) | Latest Systolic PAP (mm Hg) | PVRi baseline | Defect diameter | FU interval | comorbilities | Cause of death |
|---|---|---|---|---|---|---|---|---|---|
| (Cath)(pre) | (Echo) | WU*m2 | (mm) | (months) | |||||
| 1 | 81 | F | 88/15(37) | 44 | 3.2 | 19 | 116 | cancer | cancer |
| 2 | 67 | F | 105/31(61) | 82 | 15.7 | 21 | 43 | DM | cardiac |
| 3 | 76 | M | 61/19(35) | 57 | 3.8 | 36 | 48 | AF | stroke |
| 4 | 72 | M | 51/27(39) | 22 | 4 | 33 | 85 | DM, uremia | infection |
| 5 | 61 | F | 90/43(50) | 37 | 5.9 | 26 | 36 | DM, CAD | cardiac |
| 6 | 41 | F | 57/18(35) | 68 | 3.3 | 30 | 46 | cancer | cancer |
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