Table 5.
Evaluation and management of women with high-risk congenial heart disease. mWHO: modified World Health Organization; NYHA: New York Heart: Association; CARPREG: Cardiac Risk in Pregnancy Study; ZAHARA: Zwangerschap bij Aangeboren HARtAfwijking; CHD: congenital heart disease
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| PRECONCEPTION COUNSELING | ||
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| Estimate maternal risk | mWHO, CARPREG 2, ZAHARA | |
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| Discuss environmental risk such as diabetes, smoking, teratogenic medications etc | ||
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| Discuss fetal risk | See section on outcomes | |
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| Genetic counselling | Family history and prior pregnancy history | |
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| Discuss risk of CHD in offspring of women with CHD (6% risk of CHD in offspring if mother has CHD, 3% if father has CHD; for autosomal dominant syndromes such as 22q11 deletion or Marfan syndrome, up to 50% risk) | ||
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| Offer genetic testing when index of suspicious is high either based on phenotype (syndromic) or otherwise (non-syndromic) | ||
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| Baseline testing | ECG, echocardiogram, cardiopulmonary exercise test, liver, kidney, and thyroid function tests. Consider cross-sectional imaging in vascular disease and when echocardiographic imaging is insufficient. | |
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| Baseline O2 saturation, hemoglobin and coagulation studies, especially in cyanotic heart disease and those with thromboembolic risk | ||
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| DURING PREGNANCY | ||
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| First Trimester | Establish care with multidisciplinary team at regional adult CHD center | Cardio-obstetrics, Adult congenital Heart Disease, Obstetrics, Maternal Fetal Medicine, Anesthesia |
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| Plan trimester-wise care and follow up | ||
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| Medication reconciliation | Ensure discontinuation of teratogenic medications | |
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| Baseline testing | ECG, echocardiogram, baseline lab work as mentioned in preconception stage | |
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| Discuss lifestyle issues | Physical activity, employment, mental health, thromboembolic risk | |
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| Second Trimester | Follow up visit | Consider repeat echocardiogram as hemodynamic changes are at maximum |
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| Fetal echocardiography | ||
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| Comprehensive plan for labor, delivery, and postpartum care | Service for Delivery: Labor and Delivery with or without Telemetry versus Cardiac Care Unit | |
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| Sites for vascular access if hemodynamic monitoring in the peripartum period is planned. | ||
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| Anesthesia consults for those with possibly unstable hemodynamics, those with musculoskeletal deformities that may affect epidural placement and those with anticoagulation needs | ||
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| Cardiothoracic or Shock Team consult if mechanical circulatory support may be needed | ||
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| Social services consult if required for support | ||
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| Third Trimester | Follow-up visit | Reassess physical activity, employment, mental health, thromboembolic risk |
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| Reassess and modify as needed plan for labor, delivery, and postpartum care | ||
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| INTRAPARTUM CARE | ||
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| Induction | Consider elective induction of labor ~39 weeks | |
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| Position | Labor in right or left lateral tilt position | |
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| Second stage | Avoid Valsalva or prolonged second stage of labor. Use vacuum or forceps delivery to shorten the second stage of labor | |
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| Anesthesia | Cautious use of neuraxial anesthesia if cardiac output is preload dependent | |
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| Preferred: epidural or combined spinal epidural analgesia using narcotic with a minimal dose of local anesthetic, least chance of reducing systemic vascular resistance and worsening right to left shunting in cyanotic patients | ||
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| Cesarean Section | Cesarean delivery is usually reserved for obstetric indications | |
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| Filters in intravenous drips to avoid embolism in patients with right to left shunt | ||
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| Antibiotic prophylaxis | Reasonable to consider antibiotic prophylaxis in those with cyanotic heart disease | |
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| POSTPARTUM | ||
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| Oxytocin | Not contraindicated as postpartum hemorrhage prevention is highly important but use cautiously as hypotension and tachycardia are possible side effects | |
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| Close monitoring | Due to hemodynamic shifts first 24-48 hours are critical and close monitoring is warranted | |
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| Thromboembolic risk | Early ambulation | |
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| Postpartum visit | 6-12 weeks to assess hemodynamic state | |
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| ONGOING ADULT CONGENITAL HEART DISEASE FOLLOW-UP | ||
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