NEJM "Wrong Turn" patient.

 https://www.nejm.org/doi/full/10.1056/NEJMcps2215388

The patient is a 21-year-old woman with sickle cell trait who presented to the hospital five days after delivering her first child. She developed abdominal pain and shortness of breath following a cesarean section complicated by hemorrhagic shock and disseminated intravascular coagulation. 

On examination, she had neck pain, weakness in the left arm, jugular venous distention, crackles in both lungs, and signs of volume overload. Chest radiographs showed diffuse opacifications, and her oxygen saturation decreased. Laboratory findings included elevated troponin T and NT-proBNP levels.

ASSESSMENT

Based on the provided patient history and findings, there are several potential causes for the patient's symptoms, including:

Postoperative complications: The patient experienced hemorrhagic shock and disseminated intravascular coagulation following cesarean delivery, which could contribute to abdominal pain, dyspnea, and volume overload.

Venous thromboembolism: Given the patient's recent surgery and the presence of sickle cell trait, venous thromboembolism, such as pulmonary embolism, should be considered.

Cardiac-related causes: The patient's symptoms, including chest pain, dyspnea, and left arm weakness, raise concerns about cardiac involvement. Possible cardiac etiologies include peripartum cardiomyopathy, spontaneous coronary artery dissection (SCAD), or a congenital coronary anomaly like anomalous left coronary artery originating from the pulmonary artery (ALCAPA).

Considering the possibility of ALCAPA, it is important to evaluate further to confirm or rule out this condition. 

CT angiography can be a valuable diagnostic tool to visualize the coronary arteries and identify the abnormal origin of the left coronary artery. It can provide definitive evidence of ALCAPA and help guide treatment decisions.

[In another query, AI recommended mitigating interventions like steroids for her contrast allergy, exactly as her clinicians did in the actual case.]

If ALCAPA is confirmed, the patient's therapy options will depend on the extent of the coronary artery anomaly, collateral circulation, and associated myocardial ischemia. Treatment options for ALCAPA may include:

Surgical correction: This involves rerouting the left coronary artery to the aorta, allowing for normal blood flow and oxygenation. Different surgical techniques, such as the Takeuchi procedure or unroofing of the intramural coronary artery, can be employed based on the patient's specific anatomy and the surgeon's expertise.

Coronary artery bypass grafting (CABG): If the anomalous coronary artery cannot be surgically corrected, CABG may be considered. It involves using a healthy blood vessel, typically from the chest or leg, to create a bypass around the blocked or abnormal coronary artery, improving blood flow to the heart muscle.

The choice between surgical correction and CABG depends on various factors, including the patient's overall health, the anatomy of the anomaly, the presence of collateral circulation, and the expertise of the surgical team. It is important for the patient to be evaluated by a multidisciplinary team of cardiologists, cardiac surgeons, and interventional radiologists to determine the most appropriate treatment approach for her specific case.

It is worth noting that the discussion provided is a general overview based on the information presented. The actual management decisions should be made in consultation with the patient's healthcare team, taking into account individual patient factors and preferences.

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Tell us more about why the clinicians focuses on ALCAPA, such a rare condition, in this case as the story developed.

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The clinicians in the passage focused on ALCAPA as a potential diagnosis due to several factors and findings presented in the patient's case. Although ALCAPA is indeed rare, it is a critical condition that requires prompt diagnosis and intervention due to its potential for significant morbidity and mortality.

Several elements in the patient's presentation raised suspicion for ALCAPA:

Clinical symptoms: The patient experienced a complex postoperative course with abdominal pain, dyspnea, chest pain, and left arm weakness. These symptoms, along with signs of volume overload and elevated cardiac biomarkers, raised concerns about cardiac involvement.

Imaging findings: The transthoracic echocardiogram demonstrated reduced left ventricular ejection fraction, global hypokinesis, and dilatation of the left ventricle. The turbulent flow observed in the main pulmonary artery, as well as the late gadolinium enhancement and myocardial thinning on cardiac MRI, were suggestive of a chronic vascular process.

Electrocardiogram changes: The electrocardiogram showed T-wave inversions in multiple leads, Q waves in specific leads, and dynamic electrocardiographic changes. These findings, along with the elevated troponin levels, raised concerns about ischemia and cardiac injury.

Suspicion of other causes: The initial differential diagnosis considered other potential causes for the patient's symptoms, including postoperative complications, venous thromboembolism, peripartum cardiomyopathy, and SCAD. However, as the clinical course progressed, these diagnoses became less likely, and the focus shifted towards investigating the possibility of a congenital coronary anomaly like ALCAPA.

Considering the patient's clinical presentation, imaging findings, and the exclusion of other likely causes, the clinicians decided to pursue further evaluation for ALCAPA. This included recommending CT angiography to definitively diagnose the condition and guide appropriate treatment decisions.

While ALCAPA is rare, its potential impact on the patient's well-being and the need for timely intervention justified the focused consideration of this diagnosis in this particular case.

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Are her sickle cell disease and recent post partum state just red herrings?  If not, why is ALCAPA presenting now?  If yes, what exactly is the trigger for new onset ALCAPA Sx.

ChatGPT

The presence of sickle cell trait and the recent postpartum state are not necessarily red herrings in this case. While they may not directly cause ALCAPA, they could potentially influence the timing or severity of symptoms related to the anomaly.

ALCAPA is typically a congenital condition, meaning it is present from birth. However, the onset of symptoms can vary widely. In some cases, individuals may remain asymptomatic until adulthood or later in life, while in others, symptoms can manifest during childhood or adolescence. The exact trigger for new onset symptoms in ALCAPA is not fully understood and can vary between individuals.

In the presented case, it is possible that the physiological stressors associated with pregnancy, delivery, and the postpartum period, along with the patient's underlying sickle cell trait, may have contributed to the emergence of symptoms related to ALCAPA. These stressors could have exacerbated the myocardial ischemia caused by the abnormal coronary circulation, leading to the clinical presentation observed in the patient.

It is important to note that ALCAPA can remain undiagnosed until a triggering event occurs, such as an increase in oxygen demand or decreased oxygen supply to the myocardium. This can result in the development of symptoms and potentially life-threatening complications.

While the presence of sickle cell trait and the recent postpartum state may have played a role in the presentation of ALCAPA symptoms, it is crucial to thoroughly evaluate the patient to confirm the diagnosis and determine the most appropriate treatment approach.