Table 1
Functional classification of PH according to World Health Organization. PH: Pulmonary hypertension. Adapted from: Rich S. Primary pulmonary hypertension: executive summary. Evian, France: World Health Organization, 1998 [10].
| Functional Class | Symptom or Level of Disease |
|---|---|
| Class I | Patients with PH but without resultant limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near-syncope. |
| Class II | Patients with PH resulting in slight limitation of physical activity. Patients are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near-syncope. |
| Class III | Patients with PH resulting in marked limitation of physical activity. Patients are comfortable at rest. Less-than–ordinary activity causes undue dyspnea or fatigue, chest pain, or near-syncope. |
| Class IV | Patients with PH with the inability to carry out any physical activity without symptoms. They manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. |
Figure 1
Diagnostic algorithm and different tests recommended for pulmonary arterial hypertension according to 2015 ESC/ERS guidelines and updated clinical classification of pulmonary hypertension. Adapted from: Simonneau G et al. [8], Galiè N et al. [12], and Desai AA et al. [13]. PH: Pulmonary hypertension; PAH: Pulmonary arterial hypertension; mPAP: Mean pulmonary arterial pressure; mPAWP: Pulmonary artery wedge pressure; PVR: Pulmonary vascular resistance; WU: Woods unit; IPAH: Idiopathic pulmonary arterial hypertension; HPAH: Heritable pulmonary arterial hypertension; ECG: Electrocardiogram; TTE: Transthoracic echocardiography; TOE: Transesophageal echocardiography; RHC: Right heart catheterization; PF: Pulmonary function; BGA: Blood gas analysis.
Table 2
Determinants of PAH diseases severity and prognosis. Adapted from: Galiè N, et al. [12] BNP/NT-pro-BNP: Brain natriuretic peptide/N-terminal fragment of pro-BNP; CI: Cardiac index; CPET: Cardiopulmonary exercise testing; FC: Functional class; 6MWT: 6-minute walking test; RAP: Right atrial pressure; RV: Right ventricular; WHO: World Health Organization; RA: Right atrial.
| Determinants of Prognosis | Details | Low Risk | Medium Risk | High Risk |
|---|---|---|---|---|
| Clinical signs of RV failure evidence | Absent | Absent | Present | |
| FC [12, 16] | Classes I, II | Class III | Class IV | |
| Progression of symptoms [12, 16] | No | Slow | Rapid | |
| Exercise testing [12] | 6MWT | >440m | 165–440 m | <165 m |
| CPET | Peak oxygen consumption > 15 mL/min/kg | Peak oxygen consumption 11–15 mL/min/kg | Peak oxygen consumption < 11 mL/min/kg | |
| Clinical test [12, 15] | Echocardiographic findings | No pericardial effusion | No or minimal pericardial effusion | Pericardial effusion |
| RA area <18 cm2 | RA area 18–26 cm2 | RA area > 26 cm2 | ||
| Hemodynamics | RAP < 8mmHg CI ≥ 2.5L/min/m2 | RAP 8–14 mmHg CI 2–2.4L/min/m2 | RAP > 14 mmHg CI ≤ 2.0 L/min/m2 | |
| Biomarker test [12, 15] | BNP/NT-pro-BNP plasma levels | Normal BNP < 50 ng/L, NT-pro-BNP < 300ng/L | Elevated BNP: 50–300 ng/L, NT-pro-BNP: 300–1400 ng/L | Very elevated BNP > 300 ng/L, NT-pro-BNP > 1400 ng/L |
Figure 2
Results from COHARD-PH registry (2012–2019) in Indonesia in all registered patients (n = 1012) [7]. a) Proportion of probability of PH by echocardiography; b) Distribution by gender; c) Clinical symptoms; d) Congenital anomalies in CHD-associated PAH. CHD: Congenital heart disease; PAH: Pulmonary arterial hypertension; COHARD-PH: Congenital heart disease in adult and pulmonary hypertension; ASD: Atrial septal defect; VSD: Ventricle septal defect; PDA: Patent ductus arteriosus.
Table 3
Comparison of clinical parameters between CHD-related PAH and CHD without PAH after diagnosis by RHC (n = 614). Adapted from Dinarti LK, et al. [7]. ASD: Atrial septal defect; BNP/NT-pro-BNP: Brain natriuretic peptide/N-terminal fragment of pro-BNP; COHARD-PH: Congenital heart disease in adult and pulmonary hypertension; FC: Functional classification; IQR: Interquartile range; mPAP: Mean pulmonary arterial pressure; mRAP: Mean right atrial pressure; PDA: Patent ductus arteriosus; RHC: Right heart catheterization; VSD: Ventricle septal defect; WHO: World Health Organization.
| Characteristics of CHD Patients Based on PAH Diagnosis By RHC (N = 614) | ||
|---|---|---|
| CHD-related PAH (n = 411) | CHD without PAH (n = 203) | |
| Age at enrollment (years) (mean ± SD) | 36.4 ± 12.9 | 32.2 ± 12.0 |
| Gender (n, %) | Males: 75,18.2 | Males: 42, 20.7 |
| Females: 336, 81.8 | Females:161, 79.3 | |
| Congenital abnormalities (n, %) | ASD: 367, 89.3 | ASD: 166, 81.8 |
| VSD:17, 4.1 | VSD: 26,12.8 | |
| PDA: 21, 5.1 | PDA: 10, 4.9 | |
| 6-minute walk distance (meters) (mean ± SD) | 336.3 ± 99.7 | 393.9 ± 82.1 |
| WHO Functional class (n, %) | FC 1: 136, 34.0 | FC 1: 122, 60.4 |
| FC II: 207, 51.8 | FC II: 70, 34.7 | |
| FC III–IV: 57, 14.2 | FC III–IV: 10, 5.0 | |
| NT-pro-BNP (pg/mL)(median [IQR]) | 774.0 (242.8–2022.3) | 121.5 (57.1–218.1) |
Figure 3
Treatment algorithm for pulmonary arterial hypertension according to 2015 ESC/ERS guidelines. Adapted from: Galiè N, et al. [12], McLaughlin VV et al. [21], and Taichman DB et al. [23] PAH: Pulmonary arterial hypertension; CCB: Calcium-channel blocker; IPAH: Idiopathic pulmonary arterial hypertension; HPAH: Heritable pulmonary arterial hypertension; DPAH: Drug-induced pulmonary arterial hypertension; ET-RA: Endothelin receptor antagonist; PDE-5i: Phosphodiesterase type 5 inhibitor; PCA: Prostacyclin analog; OC: Oral combination.