Hypoplastic Left Heart Syndrome (HLHS) has recently been linked with maternal use of SSRI drugs, especially Paxil®, during pregnancy.  Hypo-, from Greek meaning “less than normal, deficient”[1], and plastic, meaning “forming; growing; changing; developing”[2], hypoplastic left heart syndrome is a birth defect characterized by the underdevelopment of the left heart.

This ailment results in the inability of the heart to pump enough blood to the body.  “As a result, the right side of the heart must maintain the circulation for both the lungs and the body.  The right ventricle can support the circulation both the lungs and the body for a while, but this extra workload eventually causes the right side of the heart to fail.”[3]

Babies with HLHS must rely on the ductus arteriosus, “a small blood vessel that connects the aorta to the pulmonary artery”[4] and the foramen ovale, a small hole between the left and right atrium, to maintain blood flowing throughout the body.[5]  While these openings usually close shortly after birth, newborns can be started on medications to keep the ductus arteriosus open.  Surgery may be required to keep the foramen ovale open.[6]

If either of these passageways are allowed to close, a baby can die quickly because blood would stop flowing continuously through the body.

Symptoms of Hypoplastic Left Heart Syndrome

“At first, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:

• Bluish (cyanosis) or poor skin color
• Cold hands and feet (extremities)
• Lethargy
• Poor pulse
• Poor suckling and feeding
• Pounding heart
• Rapid breathing
• Shortness of breath
• In healthy newborns, bluish color in the hands and feet is a response to cold (this reaction is called peripheral cyanosis).

However, a bluish color in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis). It is a sign that there is not enough oxygen in the blood. Central cyanosis often increases with crying.”[7]

Treatment for Hypoplastic Left Heart Syndrome

Treatment options for newborns suffering from HLHS are very limited.  Immediately following a diagnosis of HLHS, the newborn must be transferred to an intensive care unit, and may require a ventilator to ensure the newborn continues breathing.[8]  The drug “prostaglandin E1 is used to keep blood circulating to the body by keeping the ductus arteriosus open”[9] to stabilize the patient.

However stable the patient’s condition, babies cannot continue living in an intensive care unit idefinitely.  HLHS “always requires surgery”[10], and the heart surgery is usually performed in three stages: the Norwood operation, followed by the Glenn shunt or (hemi-Fontan) procedure, followed finally by the Fontan procedure.[11]

The Children’s Hospital of Philadelphia has published an excellent diagram of HLHS here.[12] The Norwood procedure is “the building of a new aorta,”[13] the main passageway carrying oxygen-rich blood out of the heart, using the pulmonary artery, and thus bypassing the underdeveloped left side of the heart.  Normally, the left side of the heart would take oxygen-rich blood coming from the lungs and pumps it out to the body, and the right side of the heart would only take oxygen-deficient blood from the body and pump it back to the lungs to be re-oxygenated.  In hypoplastic left heart syndrome, however, the left heart is very underdeveloped, and cannot pump oxygen-rich blood to the body.  So, the right side of the heart must also pump oxygen-rich blood to the body.

In the Norwood procedure, the underdeveloped aorta is first connected to the pulmonary artery.  The pulmonary artery would normally carry oxygen-deficient blood from the heart to the lungs, but through the Norwood procedure, it is used to instead carry oxygen-rich blood from the heart to the body.  Then, a hole must be created between the two sides of the heart, so oxygen rich blood flowing to the non-functioning left heart pump can access the functioning right side of the heart to be pumped to the body.  Finally, a “shunt” is created to ensure blood can adequately flow back to the lungs from the right heart to be re-oxygenated, while the right heart is also pumping oxygen-rich blood to the body.

The Children’s Hospital of Philadelphia provides insightful video clips explaining this operation.

[14]

After this stage, the child may go home, but must remain on medication and under the close observation of a physician.  Depending on how the child develops, the child’s physician will determine when the second surgery is to take place, usually after about 4-6 months.[15]

In the second stage of the operation, called the Glenn shunt or hemi-Fontan procedure, the shunt used in the Norwood procedure is removed, and the major veins that normally carry oxygen-deficient blood are redirected away from the heart and connected directly to the pulmonary arteries leading to the lungs for re-oxygenation.

[16]

In the third stage of the operation, the Fontan procedure, other smaller veins carrying oxygen-deficient blood to the heart are connected directly to the pulmonary arteries leading to the lungs for re-oxygenation.[17]

Once this procedure is complete, the right heart pumps oxygen-rich blood out to the body, taking over the normal role of the left heart, and the normal function of the right heart is eclipsed: oxygen-deficient blood is redirected away from the heart, and goes straight to the lungs for re-oxygenation.

[18]

Outcomes and Complications

PubMed Health, a service of the U.S. National Library of Medicine states that “If left untreated, hypoplastic left heart syndrome is fatal”[19], but reassures that the “[s]urvival after the first stage is more than 75%.”[20]

“Complications include:

• Blockage of the artificial shunt
• Chronic diarrhea (from a disease called protein losing enteropathy)
• Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
• Heart failure
• Irregular, fast heart rhythms (arrhythmias)
• Strokes and other nervous system complications
• Sudden death”[21]

We Are Here To Help

Thankfully, risk of a child being born with hypoplastic left heart syndrome can be dramatically reduced if mothers avoid SSRI drugs during pregnancy, especially Paxil®.  Because of these unreported and horrible Paxil® side effects, Paxil® lawsuits are currently being filed by families whose newborn children were injured by Paxil®.  If you believe your child’s hypoplastic left heart syndrome resulted from Paxil® or SSRI use during pregnancy, please do not hesitate to contact our law firm at (855) 452-5529 or by e-mail at justinian@dangerousdrugs.us.  We understand the pain your family has gone through, and we have the knowledge and resources needed to help you fight for justice.