Introduction:Anomalous left coronary artery arising from the pulmonary artery (ALCAPA), is a rare congenital anomaly and more so in the adult.Such malformation produces myocardial ischemia that may lead to ischemic cardiomyopathy.There have been only a few reports of ALCAPA in pregnancy. We describe a successful anesthetic management of a pregnant woman with a rare high-risk cardiovascular disease.

Case Report:A 29-year-old female with ALCAPA syndrome, diagnosed in context of a cardiac arrest, underwent surgical repair and placement of an implantable cardioverter defibrillator(ICD).Three weeks after the surgery she got pregnant.During pregnancy, the patient experienced two episodes of polymorphic ventricular tachycardia/ventricular fibrillation, one self-limited and the other requiring defibrillation.  No other complications were reported. In a multidisciplinary team discussion it was decided to performan elective C-section at 38-weeks gestation. Preoperative assessment revealed an ASAIII pregnant patient, with an enlargement of left chambers, moderate mitral insufficiency, interventricular septum hypokinesis and an ejection fraction of 53%. We performed a slowly titrated anesthetic epidural.Standard monitoring was used.It was a single try median approach technique at L3-L4 level, with fractionated administration of 75mg of Ropivacaine0.75% and 0.01mg of Sufentanil to achieve a T4 anesthesia level. During a unique moderate hypotensive episode, was required to administer slowly reduced volumes of crystalloid and 100mcg of phenylephrine.No electrocautery was used, therefore it wasn’t necessary to disable anti-tachyarrhythmia therapy.The first 24hours of the puerperium were in a post-anesthetic care unit.The puerperium was uneventful.

Discussion:Pregnancy and delivery are associated with several cardiocirculatory changes that can significantly impact underlying cardiac disease.A team of providers should develop an individualized management plan antepartum, including during pregnancy, labor and puerperium. We considered that a neuraxial anesthetic technique was the best option, because a titrated epidural technique can establish a slower onset of the surgical block with minimal hemodynamic changes. 

Learning Points: Peripartum obstetric and cardiac complications in pregnant woman with ALPACA syndrome may require urgent intervention.Interdisciplinary communication and preparation are critically important for success of both surgical and anesthetic procedures.


10 / Anesthesia for cesarean delivery in a patient with ALCAPA syndrome