What is the primary goal of immediate therapy in a 5-day-old girl with hypotension, tachycardia, tachypnea, and decreased systemic perfusion, presenting with cardiomegaly and pulmonary edema?

From the Guidelines

The answer is D. Systemic blood flow via right-to-left ductal shunting. This infant presents with classic signs of coarctation of the aorta, including decreased lower extremity blood pressure compared to upper extremities, diminished femoral pulses, and signs of heart failure (tachycardia, tachypnea, pulmonary edema) 1. In coarctation, there is narrowing of the aorta, typically just distal to the left subclavian artery, which restricts blood flow to the lower body. When the ductus arteriosus closes after birth, blood flow to the lower body becomes severely compromised. Immediate therapy aims to reopen the ductus arteriosus with prostaglandin E1 (PGE1) infusion, allowing blood to flow from the pulmonary artery to the descending aorta (right-to-left shunting), bypassing the coarctation and restoring systemic perfusion to the lower body. This explains the metabolic acidosis (low bicarbonate) and elevated lactate, which indicate tissue hypoperfusion 1. Reopening the ductus is a temporizing measure until surgical correction of the coarctation can be performed. The other options would either worsen the condition or fail to address the fundamental problem of restricted systemic blood flow to the lower body. Key points to consider in this scenario include:

• The importance of maintaining perfusion pressure above the critical point necessary for adequate organ perfusion 1
• The use of prostaglandin E1 (PGE1) infusion to reopen the ductus arteriosus and restore systemic perfusion to the lower body 1
• The need for prompt surgical correction of the coarctation to prevent long-term complications. Overall, the goal of immediate therapy is to restore systemic blood flow to the lower body and prevent further tissue hypoperfusion and organ damage.

From the Research

Immediate Therapy Goals

The primary goal of immediate therapy in this case is to increase systemic blood flow. The patient's symptoms, such as poor feeding, decreased urine output, and low blood pressure, indicate inadequate systemic perfusion.

Ductal-Dependent Cardiac Lesions

The patient's presentation suggests a ductal-dependent cardiac lesion, where the ductus arteriosus is necessary to maintain either systemic or pulmonary blood flow 2, 3, 4. In such cases, prostaglandin E1 (PGE1) therapy is commonly used to maintain patency of the ductus arteriosus.

Increasing Systemic Blood Flow

To increase systemic blood flow, the ductus arteriosus should be kept open to allow for right-to-left shunting, which would increase systemic blood flow 2, 4. This is because the patient's symptoms suggest decreased systemic perfusion, and increasing systemic blood flow would help alleviate these symptoms.

Answer Choice

Based on the evidence, the correct answer choice is:

• D. Systemic blood flow via right-to-left ductal shunting

This is because the patient's symptoms suggest a need for increased systemic blood flow, and right-to-left ductal shunting would help achieve this goal 2, 3, 4.

Key Points

• The patient's symptoms indicate inadequate systemic perfusion.
• The ductus arteriosus is necessary to maintain systemic blood flow in ductal-dependent cardiac lesions.
• Prostaglandin E1 therapy is used to maintain patency of the ductus arteriosus 2, 3, 4.
• Right-to-left ductal shunting increases systemic blood flow, which is necessary in this case.