Table 1
Anatomical-pathophysiological classification of congenital left to right shunts associated with pulmonary arterial hypertension (adapted from Simmoneau, et al.) [27].
| Classification | Congenital left to right shunts |
|---|---|
| Simple pre-tricuspid shunts |
|
| Simple post-tricuspid shunts |
|
| Combined shunts | Describe combination and define predominant defect |
| Complex congenital heart disease |
|
Table 2
PH diagnostic criteria [4].
| Echocardiography | Peak tricuspid regurgitation velocity >3.4 m/s in the absence of pulmonary outflow obstruction OR Peak tricuspid regurgitation velocity of 2.9–3.4 m/s with presence of other echocardiographic PH signs and/or some degree of right-to-left shunt |
| Catheter | mPAP > 20 mmHg and PVRi ≥ 3 WU |
Table 3
Baseline Characteristics.
| Characteristics | N=103 (%) |
|---|---|
| Total Patients | 103 (100) |
| Age at Intervention | |
| Median (months) | 44.0 |
| IQRa (months) | 78,5 |
| Mean ± SD (months) | 60.6 ± 55.2 |
| Male | 43 (42.7) |
| Home Altitude | |
| Median (feet) | 367.0 |
| IQR (feet) | 778.0 |
| Minb, Maxc (feet) | 2.5, 5177.0 |
| Type of Shunt | |
| 61 (59.2) |
| 27 (26.2) |
| 15 (14.6) |
| WHO Functional Class before correction | |
| 63 (61.2) |
| 40 (38.8) |
| History of right heart failure | 13 (12.6) |
| Severe malnutrition | 33 (47.1) |
| Down Syndrome | 13 (12.6) |
| Use of Sildenafil prior to repair | 35 (33.9) |
| Hemoglobin level (g/dL), mean ± SD | 12.1 ± 1.4 |
| Baseline mPAP, mean ± SD (mmHg) | 43.2 ± 16.1 |
| Pulmonary Vascular Resistance index (PVRi), mean± SD (WU.m2) | 2.8 ± 2.1 |
| Type of intervention | |
| 54 (52.4) |
| 49 (47.6) |
[i] a IQR, Interquartile range; b Min, Minimum value; c Max, Maximum value.
Table 4
Outcome of Repair of PAH associated CHD.
| Outcome | n = 103 |
|---|---|
| Deaths, n (%) | 9 (8.7) |
| Recovery of WHO Functional Class, n (%) | 92 (89.3) |
| Post-operative PAH, n (%) | 25 (24.3) |
| Immediate PAH crisis after repair, n (%) | 18 (17.5) |
Figure 1
Survival of patients with PAH-CHD after repair.
Figure 2
Survival of patients undergoing repair of PAH associated CHD stratified by persistence of PAH after correction.
Figure 3
Survival of patients undergoing repair of PAH associated CHD stratified by worsening of WHO Functional Class (WHO FC) after repair.
Table 5
Predictors of mortality for patients undergoing repair of PAH associated CHD.
| Variables | Hazard ratio (95% CI) | p-value |
|---|---|---|
| 1.0 (0.9–1.1) | 0.51 |
| 1.2 (0.1–5.2) | 0.86 |
| 13.4 (3.0–25.7) | 0.0002 |
| 5.3 (1.4–18.8) | 0.01 |
| 16.2 (3.6–152.6) | <0.0001 |
| 27.2 (7.2–146.4) | <0.0001 |
| 6.1 (1.6–32.8) | 0.013 |
| 34.6 (7.7–326.9) | <0.0001 |
[i] Univariate analysis with bias correction approach by the Firth penalized maximum likelihood method.
* Statistical significance at p-value < 0.05.
** PAH, pulmonary arterial hypertension.
Figure 4
Restricted mean survival time (RMST) of patients undergoing repair of PAH associated CHD grouped by persisting and reversed PAH.
Table 6
Restricted mean survival time (RMST) to disease-related mortality between group of persisting and reversed PAH after correction.
| Group | RMST (Days After Intervention) | 95% CI |
|---|---|---|
| Reversed PAH | 1194.2 | 1159.6, 1228.8 |
| Persisting PAH | 822.9 | 600.7, 1045.2 |
| Difference | –371.2 | –596.2, –146.3 |
Table 7
Difference of restricted mean survival time (RMST) between persisting PAH group and reversed PAH group adjusted for covariatesa.
| RMST (Days After Intervention) | 95% CI | P | |
|---|---|---|---|
| Difference | –476.1 | –714.4, –237,8 | <0.001 |
| Basal mPAP | 7.1 | –0.4, 14.6 | 0.06 |
| Age at Intervention | 1.9 | –0.1, 4.1 | 0.06 |
| PVRi | –60.3 | –99.7, –20.8 | 0.003 |
[i] a Estimates are adjusted to basal mPAP (mean Pulmonary Artery Pressure), age at intervention and PVRi (Pulmonary Vascular Resistance Index).