Left ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy that is characterized by prominent trabeculations of the left ventricle and a spongy appearance of the myocardium.1 It is thought to be due to a developmental failure of the heart to form the compact myocardium during the later embryonic stages of cardiac development.2 The clinical presentation can be protean and can include congestive heart failure, atrial and ventricular arrhythmias, and thromboembolic disease. The first presentation can occur during pregnancy.3 We describe the case of a parturient with LVNC who presented for cesarean delivery. She had worsening heart failure that was complicated by an acute pulmonary hypertensive crisis after methylergonovine administration for postpartum hemorrhage.
The patient gave permission for the authors to publish this report.
A 31-year-old woman, gravida 4, para 1, with a singleton intrauterine pregnancy at 34 weeks gestation presented for cesarean delivery. She had a history of LVNC, poor left ventricular (LV) compliance, and pulmonary hypertension with worsening heart failure.
The patient had been diagnosed with LVNC after her first pregnancy and delivery. The pregnancy was well tolerated but was complicated by persistent postpartum swelling requiring diuretics. A cardiac workup revealed moderate pulmonary hypertension at rest (pulmonary artery pressure of 60/22 mm Hg with a mean of 36 mm Hg), severe pulmonary hypertension with exercise (pulmonary arterial pressure of 90/30 mm Hg with a mean of 60 mm Hg), and severe LVNC. The right ventricle (RV) did not appear to be noncompacted. She underwent a repeat cardiac catheterization 3 years later that showed no significant change in her hemodynamics. She had remained asymptomatic and continued to exercise on a daily basis. She subsequently became pregnant in 2013 and decided to continue with the pregnancy, despite counseling to the contrary by her physicians.
The patient was asymptomatic until 30 weeks gestation, at which point she began experiencing dyspnea and orthopnea. Transthoracic echocardiography (TTE) at that time revealed moderately decreased LV systolic function, an LV ejection fraction of 38%, mild decreased RV function, mild-to-moderate mitral regurgitation, and mild pulmonary hypertension (pulmonary artery systolic pressure of 41 mm Hg). The dyspnea worsened, and a decision was made to perform a cesarean delivery at 34 weeks gestation.
Preoperatively, the patient was admitted to the cardiac care unit for monitoring. A 9F introducer sheath and pulmonary artery catheter were placed in the right internal jugular vein to monitor pulmonary artery pressures and RV function that could be affected by the fluid shifts and increased preload resulting from the delivery of the baby and the contracting uterus. Preoperative TTE showed worsening cardiac function: an LV ejection fraction of 23%, moderately to severely decreased RV function, mild-to-moderate mitral regurgitation, and severe pulmonary hypertension (pulmonary systolic pressure of 73 mm Hg).
The delivery was scheduled to be performed in the cardiac operating rooms (ORs), with extracorporeal life support equipment and cardiothoracic surgeons on standby. When the patient arrived at the OR, a left radial artery line was placed, and dobutamine infusion was initiated. General anesthesia was induced with etomidate 20 mg and succinylcholine 120 mg, and the patient’s trachea was atraumatically intubated. Anesthesia was maintained with 0.3% isoflurane, remifentanil (0.1 μg/kg/min), and incremental boluses of fentanyl to a total dose of 500 μg. The patient was monitored with transesophageal echocardiography (TEE) throughout the procedure. Initial TEE examination showed no changes from the previous day’s TTE.
Two minutes after incision, a healthy male infant was delivered with Apgar scores of 8 at both 1 and 5 minutes. Oxytocin 20 units/h was started, as per routine, after delivery of the placenta. The delivery was complicated by uterine atony. Despite attempts by the obstetricians to halt the bleeding by using uterine massage and compression sutures, bleeding was not controlled, and methylergonovine 0.2 μg was injected IM. Although some improvement was seen, bleeding continued and a second dose of methylergonovine 0.2 μg was given 5 minutes later, which improved uterine tone. After the second dose of methylergonovine was injected, mean arterial blood pressure of the patient decreased to the low 70s, and her mean pulmonary artery pressure increased from the 60s to the 80s. TEE showed worsening RV failure. Ten 16-μg boluses of epinephrine were given over 2 minutes, and a vasopressin infusion of 2 units/h was initiated, which improved arterial and pulmonary pressures.
The operation was completed 50 minutes after incision, and the patient’s trachea was extubated 10 minutes later. She remained hemodynamically stable in the OR for another 30 minutes and was subsequently transported to the cardiothoracic surgical intensive care unit for further monitoring. Total estimated blood loss was 1200 mL.
Inotropic medications were gradually discontinued on postoperative day 1. Postoperative medical management included administering carvedilol and enoxaparin and inducing diuresis with furosemide, and implantable cardioverter-defibrillator placement was considered. Three days after the operation, TTE revealed that the LV ejection fraction had increased to 40%, the RV was moderately dysfunctional, and the pulmonary artery systolic pressure was 63 mm Hg. The patient was discharged to home in stable condition 4 days postoperatively. The patient declined placement of an implantable cardioverter defibrillator.
Patients with LVNC may be asymptomatic or they may present with signs of heart failure.3 The normal physiologic changes that accompany pregnancy, such as increased blood volume and cardiac output, are not well tolerated by patients with LVNC and may precipitate symptoms of heart failure.4 The difficulty is exacerbated in patients who also have pulmonary arterial hypertension, which has been associated with maternal mortality as high as 56%.5 Pulmonary hypertension frequently occurs in patients with LVNC as the systolic and diastolic heart dysfunction leads to increased pulmonary arterial pressures. This may then result in RV dysfunction, as well. Such patients require a multidisciplinary approach to delivery planning. Obstetricians, cardiologists, and anesthesiologists were involved in this patient’s care from early pregnancy.
Mode of delivery in the parturient with cardiac disease is determined by obstetrical indications and maternal functional status.6 Vaginal delivery is appropriate for a parturient with well-compensated disease and has the benefits of greater hemodynamic stability, decreased blood loss, and minimal surgical stress. Our patient had progressive worsening of her cardiac status, so cesarean delivery at 34 weeks was recommended.
Anesthetic options for cesarean delivery include neuraxial and general anesthesia. Incrementally dosed epidural anesthesia with slow titration of local anesthetics serves to decrease preload and afterload and may help patients tolerate the increased blood volume from uterine autotransfusion after delivery.7 Epidural anesthesia also has the benefit of decreased postoperative pain and, therefore, limits sympathetically medicated cardiac stress.
In this case, we selected general anesthesia because our patient had already manifested symptoms of decompensated cardiac failure and was unable to lie flat. We used a remifentanil infusion and small boluses of fentanyl, which provided stable hemodynamics and blunted the sympathetic response to pain. General anesthesia also has the additional advantage of allowing hemodynamic monitoring with TEE.8
Uterine atony and the concomitant need for rapid infusion of fluid and blood products are poorly tolerated in women with LVNC. Additionally, methylergonovine causes rapid and sustained contractions by acting directly on both uterine and vascular smooth muscle.9 This may lead to systemic vasoconstriction. In fact, several cases of acute myocardial infarction have been reported after injecting methylergonovine.10,11 Furthermore, methylergonovine has been associated with increased pulmonary artery pressures12 and should be used with extreme caution in patients with pulmonary hypertension.
Methylergonovine may also have some actions on the central nervous systems because it is both an agonist and antagonist at the serotonin and dopamine receptors and therefore may be useful for the treatment of migraine headaches. The hemodynamic consequences of these receptor interactions are unknown.13 Other uterotonic drugs, such as carboprost and misoprostol, were alternative options. However, carboprost also has cardiac effects, including systemic and pulmonary hypertension; therefore, with regard to avoiding cardiac effects, it has no advantage over methylergonovine.14
Although misoprostol has no cardiac side effects that have been described, its efficacy as an uterotonic has not been fully defined. Its prophylactic use to prevent uterine atony has been described with rectal or buccal administration after birth. Some studies have found it to be as effective15 as other uterotonic drugs, whereas others have not.16 Theoretically, because uterotonics such as methylergonovine and carboprost have known significant cardiovascular side effects, the cardiac patient may be an ideal candidate for prophylactic misoprostol administration. In this case, we only chose to administer methylergonovine after other options for halting the bleeding, including uterine massage, retention sutures, and oxytocin administration, were unsuccessful. Continued bleeding necessitating rapid transfusion posed substantial risks for this patient, so we chose the drug most familiar to us (misoprostol), while preparing to treat pulmonary or systemic hypotension if it occurred.
Although methylergonovine has been associated with increased pulmonary artery pressures,12 we believe that this is the first report of an acute pulmonary hypertensive crisis with RV failure precipitated by methylergonovine. We cannot definitively state that the crisis was precipitated by the increased preload caused by the contracting uterus, but the timing of events suggests that methylergonovine was the cause. Furthermore, although methylergonovine is known to cause systemic vasoconstriction as well, the arterial blood pressure of our patient significantly decreased because the pulmonary vasoconstriction caused an already struggling RV to fail. In patients with a failing RV, an aggressive approach is necessary. We used boluses of epinephrine for further inotropic support and vasopressin to maintain myocardial perfusion pressure. Vasopressin was chosen over other vasopressors such as norepinephrine because it does not cause a concomitant increase in pulmonary pressures.
The parturient with LVNC presents a unique challenge to anesthesiologists. A complete understanding of the pathophysiology of such patients, along with a multidisciplinary team approach, is critical for a successful outcome.
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