What is the management of cyanotic and non-cyanotic congenital heart defects?

Management of Cyanotic and Non-Cyanotic Congenital Heart Defects

General Management Principles

All adults with congenital heart disease (CHD), particularly cyanotic patients, should be managed at specialized centers with staff experienced in complex congenital anatomy and should be seen at least annually by an ACHD (Adult Congenital Heart Disease) specialist. 1

---

Cyanotic Congenital Heart Disease Management

Initial Assessment and Monitoring

Cyanotic patients require specialized care focusing on preventing complications from chronic hypoxemia, erythrocytosis, and hyperviscosity. 1

• Right-to-left intracardiac or extracardiac shunts result in hypoxemia, erythrocytosis, and cyanosis 1
• Survival is determined by the underlying CHD type and medical complications of cyanosis 1
• Annual follow-up with ACHD specialist is mandatory 1

Hematologic Management

Therapeutic phlebotomy should ONLY be performed when hemoglobin exceeds 20 g/dL AND hematocrit exceeds 65% with associated symptoms of hyperviscosity (headache, fatigue) in the absence of dehydration or anemia. 1

• Most cyanotic patients have compensated erythrocytosis with stable hemoglobin requiring no intervention 1
• When phlebotomy is indicated, remove 1 unit of blood with equal volume replacement of dextrose or saline 1
• Repeated routine phlebotomies are contraindicated due to risk of iron depletion, decreased oxygen-carrying capacity, and stroke 1
• Iron deficiency can destabilize erythropoiesis and worsen outcomes 1

Hemostatic Considerations

• Hemostatic abnormalities occur in up to 20% of cyanotic patients due to platelet dysfunction and clotting factor deficiencies 1
• Anticoagulants and antiplatelet agents should be used with extreme caution 1
• Common bleeding manifestations include epistaxis, gingival bleeding, menorrhagia, and pulmonary hemorrhage 1

Neurological Complications Prevention

Meticulous attention to intravenous line management is essential to prevent paradoxical air embolism, which can cause cerebral emboli. 1, 2

• Brain abscess should be suspected in cyanotic patients with headache, fever, and new neurological symptoms 1
• Thromboembolic events can occur with atrial tachycardia or atrial stasis associated with transvenous pacing leads 1

Travel and Lifestyle Modifications

Cyanotic patients must drink non-alcoholic and non-caffeinated fluids frequently during long-distance flights to avoid dehydration. 1

• Only use pressurized commercial airplanes 1
• Supplemental oxygen may be considered for long-distance flights 1
• Residence at high altitude is detrimental and should be avoided 1
• Competitive sports should be avoided 1
• Early ambulation during hospitalization prevents venous stasis and thrombophlebitis 1

Cardiac Device Management in Cyanotic Patients

Epicardial pacemaker and device lead placement must be performed in all cyanotic patients with intracardiac shunts who require devices. 1

• Endocardial leads are contraindicated due to risk of paradoxical embolism 1
• Device placement should only occur at centers familiar with unusual anatomy of congenital heart defects 1
• Complete echocardiographic evaluation and review of all surgical records is mandatory before device placement 1

Surgical Considerations

Preoperative evaluation by an ACHD cardiologist and cardiac anesthesiologist is mandatory before any operation in cyanotic patients, especially those with pulmonary arterial hypertension. 1

• Cyanotic patients are at high risk during any hospitalization or operation 1
• Scoliosis surgery may be contraindicated in cyanotic patients with PAH 1
• Medication adjustment accounting for cyanosis is required during hospitalization 1

---

Non-Cyanotic Congenital Heart Disease Management

Arrhythmia Management

Catheter ablation procedures and pacemaker placement for ACHD patients must be performed at centers where staff is experienced with complex anatomy and distinctive arrhythmia substrates. 1

• Cardiac arrhythmias are a major source of morbidity and mortality for ACHD patients 1
• Most difficult cases involve patients with prior intracardiac repairs, especially when performed late in life 1
• Decisions regarding tachycardia management should consider repairable hemodynamic issues that might favor surgical or catheter-based approaches 1

Atrioventricular Block Management

Permanent pacemaker implantation is indicated for postoperative advanced second- or third-degree AV block that persists at least 7 to 10 days after cardiac surgery. 1

• Surgical repair of CHD may cause direct trauma to AV conduction tissues 1
• In more than half of cases, AV block is transient and conduction recovers within 7-10 days 1
• Patients with congenitally corrected TGA (CCTGA) and AV septal defect (especially with Down syndrome) require periodic assessment with serial ECGs and Holter monitoring 1

Specific Lesion Management

Tetralogy of Fallot

Primary repair between 6 and 18 months of age is current standard practice, with peri-operative mortality of 1%. 1

• Repair involves VSD closure and relief of RVOTO with infundibular resection and pulmonary valvotomy 1
• Many patients require additional RVOT or transannular patches 1
• 35-year survival after repair is 85% 1
• Severe chronic pulmonary regurgitation eventually leads to symptomatic RV dilation and dysfunction 1

Transposition of Great Arteries

Arterial switch (Jatene) procedure is the treatment of choice for TGA with intact ventricular septum or with VSD. 3, 4

• Rastelli procedure is required for patients with both VSD and pulmonary stenosis 3, 4
• Prostaglandin E1 infusion and/or balloon atrial septostomy may be needed prior to corrective surgery 3, 4

Tricuspid Atresia

Initial palliation with modified Blalock-Taussig shunt or pulmonary artery banding is required, followed by staged Fontan procedure. 3, 4

• Staged approach includes bidirectional Glenn followed by fenestrated Fontan with extra-cardiac conduit 3, 4

---

Common Pitfalls to Avoid

• Never perform routine phlebotomies in cyanotic patients - this increases stroke risk and causes iron deficiency 1, 2
• Never place endocardial pacing leads in cyanotic patients with intracardiac shunts - risk of paradoxical embolism mandates epicardial approach 1
• Never allow dehydration in cyanotic patients - this precipitates hyperviscosity complications 1, 2
• Never perform complex procedures at non-specialized centers - outcomes are significantly worse without ACHD expertise 1