Key Points
- Stabilize ABCs; begin targeted evaluation without delaying life-saving therapy.
- Use system-specific risk tools to guide testing and disposition.
- Order high-yield tests first; escalate imaging when indicated.
- Start evidence-based initial therapy and reassess frequently.
Algorithm
- Primary survey and vitals; IV access and monitors.
- Focused history/physical; identify red flags and likely etiologies.
- Order system-appropriate labs and imaging (see Investigations).
- Initiate guideline-based empiric therapy (see Pharmacology).
- Reassess response; arrange consultation and definitive management.
Clinical Synopsis & Reasoning
For Adult Congenital Heart Disease Primary Care Essentials, frame the differential by acuity and pathophysiology, then align diagnostics to the leading hypotheses. Prioritize stabilization while obtaining high‑yield studies such as EKG (Rhythm/ischemia), Troponin (Myocardial injury), CXR (Pulmonary edema/size), BMP/Mg2+ (Electrolytes/renal). Incorporate bedside imaging and targeted labs to define severity and identify complications; synthesize results with clinical trajectory to refine the working diagnosis and disposition needs.
Treatment Strategy & Disposition
Initiate disease‑directed therapy alongside supportive care, titrating to objective response. Pharmacologic options commonly include Aspirin, P2Y12 inhibitor, Heparin/LMWH, Beta-blocker. Use validated frameworks (e.g., Examples — ACHD Lesions & Pregnancy Risk (mWHO)) to guide escalation and site of care. Address precipitating factors, de‑escalate empiric therapies with data, and arrange follow‑up for monitoring and risk‑factor modification; admit patients with instability, high risk of deterioration, or needs for close monitoring.
Epidemiology / Risk Factors
- Atherosclerotic risk (HTN, DM, HLD, smoking)
- Age/family history of premature CAD
Investigations
| Test | Role / Rationale | Typical Findings | Notes |
|---|---|---|---|
| EKG | Rhythm/ischemia | ST-T changes/arrhythmia | Serial |
| Troponin | Myocardial injury | Dynamic rise/fall | Trend |
| CXR | Pulmonary edema/size | Cardiomegaly/edema | |
| BMP/Mg2+ | Electrolytes/renal | Derangements | |
| CBC/Coags | Bleeding risk | Abnormal/INR |
Examples — ACHD Lesions & Pregnancy Risk (mWHO)
| Lesion | mWHO Class | Notes |
|---|---|---|
| Repaired simple ASD/VSD | II | Low‑moderate risk |
| Fontan circulation | III–IV | High risk; expert care |
| Severe systemic ventricular dysfunction | IV | Pregnancy contraindicated |
| Marfan with aortic dilation | IV | Contraindicated |
| Bicuspid aortopathy (moderate) | III | Close imaging surveillance |
Pharmacology
| Medication | Mechanism | Onset | Role in Therapy | Limitations |
|---|---|---|---|---|
| Acetaminophen | Analgesic/antipyretic | Hours | Symptom control as appropriate | Hepatotoxicity (overdose) |
| Ondansetron | 5-HT3 antagonism | Minutes | Antiemesis if needed | QT prolongation |
Prognosis / Complications
- Prognosis by ischemic burden/LV function
- Arrhythmias and HF are complications
Patient Education / Counseling
- Explain red flags and when to seek emergent care.
- Reinforce medication adherence and follow-up plan.
Primary Care Notes
Ensure clear documentation, vaccination updates, and dental care. Coordinate with ACHD specialists for non‑cardiac procedures, anesthesia planning, and transition of care from pediatric services.
References
- 2018 AHA/ACC ACHD Guideline — Link