Below is a comprehensive structured overview of all four syndromes based on retrieved sources and established clinical knowledge.
I. SYNDROME OF SYSTEMIC HYPERTENSION
Definition
Systemic (arterial) hypertension is a sustained elevation of blood pressure (BP) to ≥140/90 mmHg measured on at least 2 separate occasions. It is divided into primary (essential) (~90–95%) and secondary (~5–10%) forms.
Complaints (Symptoms)
Most patients are asymptomatic for years ("the silent killer"). When symptoms occur:
| Category | Symptoms |
|---|
| Headache | Occipital, typically morning, pulsating |
| Neurological | Dizziness, tinnitus, visual blurring ("spots"), impaired concentration |
| Cardiac | Palpitations, chest discomfort, dyspnea on exertion |
| General | Fatigue, epistaxis, flushing |
| Hypertensive urgency/emergency | Severe headache, nausea/vomiting, confusion, visual loss, focal neurological deficits |
Classification
By BP Level — ACC/AHA 2017 Guidelines
| Category | Systolic (mmHg) | | Diastolic (mmHg) |
|---|
| Normal | < 120 | and | < 80 |
| Elevated | 120–129 | and | < 80 |
| Stage 1 HTN | 130–139 | or | 80–89 |
| Stage 2 HTN | ≥ 140 | or | ≥ 90 |
| Hypertensive Crisis | > 180 | and/or | > 120 |
Note: ESC/ESH 2018 retains the threshold of ≥140/90 mmHg for Grade 1 hypertension.
ESC/ESH Grading
| Grade | SBP (mmHg) | DBP (mmHg) |
|---|
| Grade 1 (Mild) | 140–159 | 90–99 |
| Grade 2 (Moderate) | 160–179 | 100–109 |
| Grade 3 (Severe) | ≥ 180 | ≥ 110 |
| Isolated systolic HTN | ≥ 140 | < 90 |
By Etiology
- Primary (Essential): No identifiable cause; multifactorial (genetic, environmental)
- Secondary: Renal (renovascular, parenchymal), endocrine (primary hyperaldosteronism, pheochromocytoma, Cushing's, hyperthyroidism), coarctation of aorta, obstructive sleep apnea, drug-induced
By Target Organ Damage (Risk Stratification)
- Stage A – No TOD, no CV risk factors
- Stage B – Risk factors present, no TOD
- Stage C – TOD/CVD present (LVH, retinopathy, nephropathy, atherosclerosis)
Diagnostic Criteria
- Office BP ≥ 140/90 mmHg on ≥ 2 readings at ≥ 2 visits
- 24-hour ABPM (ambulatory): Mean ≥ 130/80 mmHg (daytime ≥ 135/85, nighttime ≥ 120/70)
- Home BP monitoring (HBPM): ≥ 135/85 mmHg
- White-coat hypertension excluded with ABPM/HBPM
- Workup includes: CBC, urinalysis, BMP (creatinine, K⁺), fasting glucose/lipids, ECG (LVH), echocardiography, fundoscopy, renal ultrasound if secondary cause suspected
Hypertensive Urgency vs Emergency
| Feature | Urgency | Emergency |
|---|
| BP | >180/120 | >180/120 |
| Target organ damage | Absent | Present (AKI, encephalopathy, pulmonary edema, dissection, stroke) |
| Management | Oral agents, lower over hours | IV agents, ICU, lower within minutes–1 hour |
II. SYNDROME OF SYSTEMIC HYPOTENSION
Definition
Arterial hypotension is a sustained or symptomatic reduction in systolic BP < 90 mmHg and/or diastolic BP < 60 mmHg, or a fall sufficient to cause end-organ hypoperfusion.
Complaints (Symptoms)
| System | Symptoms |
|---|
| CNS | Lightheadedness, dizziness, presyncope, syncope, blurred vision, cognitive slowing |
| Cardiovascular | Palpitations (reflex tachycardia), chest pain (if coronary hypoperfusion) |
| General | Weakness, fatigue, cold extremities, pallor, diaphoresis |
| Orthostatic | Symptoms triggered by standing (within 3 min): dizziness, "black-out," falls |
| Postprandial | Dizziness/syncope 15–60 min after eating |
Classification
| Type | Definition | Key Causes |
|---|
| Primary (Constitutional) Hypotension | Chronic low BP without identifiable cause; asthenic body habitus, more common in young women | Idiopathic; constitutional tendency |
| Orthostatic (Postural) Hypotension | ↓ SBP >20 mmHg or ↓ DBP >10 mmHg within 3 min of standing | Dehydration, autonomic neuropathy (DM, Parkinson's), medications (antihypertensives, diuretics, α-blockers), Addison's disease |
| Postprandial Hypotension | BP fall ≥20 mmHg within 2 h after eating | Elderly, autonomic dysfunction |
| Neurally Mediated (Vasovagal/Reflex) Syncope | Reflex-mediated vasodilation and/or bradycardia | Pain, prolonged standing, emotional stress |
| Secondary Hypotension | Due to identifiable disease | Cardiogenic shock, Addison's, hypothyroidism, sepsis, severe hemorrhage, pulmonary embolism |
| Drug-Induced | Iatrogenic | Antihypertensives, diuretics, nitrates, antidepressants |
| Orthostatic Hypotension with autonomic failure | Associated with absent compensatory tachycardia | DM, Parkinson's, MSA (Shy-Drager) |
Diagnostic Criteria
Per Harrison's Principles of Internal Medicine (p. 6764):
"Orthostatic hypotension is defined by a fall in systolic pressure >20 mmHg or in diastolic pressure >10 mmHg in response to assumption of the upright posture from a supine position within 3 min. There may also be a lack of a compensatory tachycardia, an abnormal response that suggests autonomic insufficiency."
General Diagnostic Criteria for Hypotension
| Criterion | Threshold |
|---|
| Systolic BP | < 90 mmHg |
| Diastolic BP | < 60 mmHg |
| Mean Arterial Pressure | < 70 mmHg |
| Orthostatic drop (SBP) | > 20 mmHg on standing |
| Orthostatic drop (DBP) | > 10 mmHg on standing |
Diagnostic Workup
- Tilt-table test – gold standard for vasovagal/neurally mediated syncope
- Active stand test – orthostatic hypotension
- 24-hour ABPM – to identify pattern
- Holter monitor – if arrhythmia-related syncope suspected
- Laboratory: cortisol, ACTH stimulation (Addison's), TSH, CBC, BMP
- Echocardiography – if cardiogenic cause suspected
III. SYNDROME OF PULMONARY HYPERTENSION
Definition
Pulmonary hypertension (PH) is defined hemodynamically as a mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest (recent ESC/ERS 2022 updated threshold: > 20 mmHg), measured by right heart catheterization (RHC).
Complaints (Symptoms)
| Stage | Symptoms |
|---|
| Early | Exertional dyspnea (most common, insidious onset), fatigue, reduced exercise tolerance |
| Intermediate | Exertional chest pain (anginal — RV ischemia), exertional syncope/presyncope, palpitations |
| Advanced | Dyspnea at rest, peripheral edema (ankle swelling), ascites, right upper quadrant pain (hepatic congestion), cyanosis |
| Associated with underlying disease | Hemoptysis (in PAH, CTEPH), Raynaud's, dry cough |
Key hallmark: dyspnea out of proportion to the underlying lung or cardiac disease.
Classification (WHO/ESC 2022 — 5 Groups)
| Group | Etiology |
|---|
| Group 1 — PAH | Idiopathic, heritable (BMPR2 mutation), drug/toxin-induced (anorexigens, meth), connective tissue disease (SSc, SLE, MCTD), congenital heart disease, portopulmonary, HIV, schistosomiasis |
| Group 2 — Left Heart Disease | HFpEF, HFrEF, valvular disease (mitral stenosis, aortic stenosis) |
| Group 3 — Lung Disease/Hypoxia | COPD, ILD, sleep-disordered breathing, high altitude |
| Group 4 — CTEPH | Chronic thromboembolic pulmonary hypertension (post-PE) |
| Group 5 — Miscellaneous/Unclear | Hematologic disorders (PV, ET), sarcoidosis, metabolic disorders, fibrosing mediastinitis |
WHO Functional Class (FC) — Severity of PH Symptoms
| FC | Description |
|---|
| I | No limitation; ordinary activity causes no symptoms |
| II | Slight limitation; comfortable at rest; ordinary activity causes dyspnea/fatigue |
| III | Marked limitation; comfortable at rest; less-than-ordinary activity causes symptoms |
| IV | Unable to perform any physical activity; symptoms at rest; signs of RV failure |
Diagnostic Criteria
Hemodynamic Thresholds (Right Heart Catheterization)
| Parameter | Threshold | Type |
|---|
| mPAP | > 20 mmHg | All PH |
| mPAP | ≥ 25 mmHg | Classic threshold (pre-2022) |
| PAWP | ≤ 15 mmHg | Pre-capillary PH (Groups 1, 3, 4, 5) |
| PAWP | > 15 mmHg | Post-capillary PH (Group 2) |
| PVR | > 2 Wood Units | Pre-capillary PH component |
Diagnostic Algorithm
- Echocardiography – screening tool; estimate RVSP via tricuspid regurgitation jet velocity:
- Low probability: TRV ≤ 2.8 m/s
- Intermediate: TRV 2.9–3.4 m/s
- High probability: TRV > 3.4 m/s or multiple indirect signs
- Right Heart Catheterization (RHC) – gold standard (mandatory before initiating PAH-specific therapy)
- V/Q scan – CTEPH screening (Group 4)
- HRCT chest – lung disease, ILD (Group 3)
- PFTs, 6-minute walk test (6MWT), BNP/NT-proBNP
- Connective tissue disease serologies (ANA, anti-Scl70, anti-centromere)
IV. SYNDROME OF PULMONARY HEART (COR PULMONALE)
Definition
Cor pulmonale is right ventricular hypertrophy (RVH) and/or dilation and dysfunction secondary to pulmonary hypertension caused by diseases of the lung parenchyma, pulmonary vasculature, or thoracic cage — excluding left heart disease or congenital heart disease.
- Acute cor pulmonale: Sudden RV pressure overload (e.g., massive pulmonary embolism)
- Chronic cor pulmonale: Gradual RVH/dilation due to chronic lung disease (COPD most common)
Complaints (Symptoms)
| Phase | Symptoms |
|---|
| Compensated | Exertional dyspnea, fatigue, cough (from underlying lung disease), reduced exercise tolerance |
| Decompensated (Right Heart Failure) | Peripheral edema (bilateral, pitting), weight gain, ascites, right upper quadrant pain/fullness (hepatomegaly), anorexia, nausea |
| Advanced | Orthopnea (due to ascites), syncope on exertion, central cyanosis, hemoptysis |
| Physical exam findings | JVD, hepatojugular reflux, loud P₂, tricuspid regurgitation murmur, S3/S4 (right-sided), Kussmaul's sign, parasternal heave |
Classification
| Type | Mechanism | Common Causes |
|---|
| Acute | Sudden RV pressure overload | Massive PE, ARDS, severe pneumonia |
| Chronic | Longstanding PH → RVH → RV dilation | COPD (~50%), ILD, obstructive sleep apnea, kyphoscoliosis, CTEPH, neuromuscular disease |
Diagnostic Criteria
Clinical Criteria
- Evidence of RVH/RV dysfunction on imaging
- Evidence of pulmonary hypertension as the underlying cause
- Exclusion of left heart disease as the primary etiology
ECG Findings (RVH Pattern)
| Finding | Significance |
|---|
| Right axis deviation (> +110°) | RVH |
| R/S ratio > 1 in V1, R > 7 mm in V1 | RVH |
| S1Q3T3 pattern | Acute cor pulmonale (PE) |
| P pulmonale (P > 2.5 mm in II, tall peaked) | Right atrial enlargement |
| Incomplete/complete RBBB | RV strain |
| ST depression + T-wave inversion V1–V4 | RV strain |
Echocardiography (Key Findings)
- RV dilation and hypertrophy (RV wall > 5 mm)
- Paradoxical interventricular septal motion ("D-sign" on short-axis)
- Elevated RVSP (via TR jet)
- Dilated right atrium, dilated IVC (> 2.1 cm, < 50% collapse with inspiration → elevated RAP)
- Tricuspid regurgitation
- Reduced RV fractional area change (FAC) and TAPSE (< 17 mm = RV dysfunction)
Chest X-Ray
- Enlarged main pulmonary artery and hilar vessels ("pruning" peripherally)
- Right ventricular enlargement (filling of retrosternal space on lateral view)
- Evidence of underlying lung disease (hyperinflation in COPD, reticular pattern in ILD)
Laboratory Markers
| Test | Finding |
|---|
| BNP / NT-proBNP | Elevated (RV wall stress) |
| Polycythemia (CBC) | Chronic hypoxia |
| ABG | Hypoxemia ± hypercapnia |
| LFTs | Elevated in hepatic congestion |
Right Heart Catheterization (Definitive)
- mPAP > 25 mmHg
- Normal PAWP (≤ 15 mmHg) — pre-capillary origin
- Elevated RAP and RV end-diastolic pressure
- Reduced cardiac output in decompensated cor pulmonale
Summary Comparison Table
| Feature | Systemic HTN | Systemic Hypotension | Pulmonary HTN | Cor Pulmonale |
|---|
| Key definition | BP ≥140/90 mmHg | SBP <90, DBP <60 mmHg | mPAP >20 mmHg (RHC) | RVH/RV dysfunction from pulmonary cause |
| Primary symptom | Often asymptomatic → headache | Dizziness, syncope | Exertional dyspnea | Dyspnea + RV failure signs |
| Diagnostic gold standard | Office/ABPM BP measurement | Tilt table test (vasovagal); RHC excluded | Right heart catheterization | Echo + RHC |
| Key ECG finding | LVH (Sokolow-Lyon, Cornell) | None specific | RVH pattern | RVH, P pulmonale, S1Q3T3 |
| Biomarker | None specific | Cortisol (if Addison's) | BNP/NT-proBNP | BNP/NT-proBNP, polycythemia |
| Classification basis | BP level (Grades 1–3) | Type (orthostatic, vasovagal, secondary) | WHO Groups 1–5 | Acute vs. Chronic |
Sources: ACC/AHA 2017 High Blood Pressure Guidelines (p. 64); Harrison's Principles of Internal Medicine, 21st Ed. (p. 6764); ESC/ERS 2022 Pulmonary Hypertension Guidelines; WHO/Dana Point Classification.