Cirugía de síndrome del corazón izquierdo hipoplásico (SCIH)

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Norton Children’s Heart Institute

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Hypoplastic left heart syndrome (HLHS) is an abnormality of the heart that a baby is born with. The left side of the heart is smaller and weaker than it should be. HLHS surgery is required to create new pathways for the heart to pump oxygen-rich blood through the body.

Since the left side of the newborn’s heart doesn’t pump enough blood, the right side of the heart has to work harder but won’t be getting enough oxygen from the lungs.

A baby born with HLHS often has an atrial septal defect (ASD) — a hole between the ventricles that actually helps their heart function. The hole allows oxygenated blood from the left ventricle mix with the blood being pumped out to their body.

Babies born with HLHS but without this hole will need an emergency intervention called a balloon septostomy shortly after birth to create such a hole.

Another hole — the patent ductus arteriosus (PDA) — is normal and usually closes within the first few days of life. In a baby with HLHS, this hole also helps mix the oxygenated blood and needs to be kept open. Prostaglandin is a medication frequently given to babies with HLHS after birth to keep the PDA open. The cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are prepared and experienced to act swiftly to keep the PDA and the ASD open until the heart is repaired with HLHS surgery.

It wasn’t long ago that babies born with HLHS didn’t survive. Using the latest techniques, the board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to effectively rebuild a baby’s circulatory system with HLHS surgery, helping them to potentially live a full and productive life.

Diagnóstico del síndrome del corazón izquierdo hipoplásico

El SCIH se puede ver en las ecografías durante el embarazo. Un ecocardiograma fetal proporciona más información y le indica al equipo de parto cómo prepararse para la atención después del parto.

Las pruebas comunes que se usan para obtener más información sobre el corazón de un recién nacido incluyen:

  • Echocardiogram (ultrasound images and videos of the heart)
  • Radiografía de tórax, una imagen del corazón y los órganos circundantes
  • Electrocardiogram (ECG or EKG), a recording of the heart’s electrical activity
  • Pulse oximetry (measuring the oxygen in blood on a fingertip or toe)

Cirugía de SCIH

HLHS most often is diagnosed through ultrasound before the baby’s birth. The cardiothoracic surgeons at Norton Children’s Heart Institute are poised to stabilize the newborn immediately while more diagnostic tests are performed.

In addition to administering prostaglandin to keep the PDA open, doctors will use medications and mechanical ventilation strategies (breathing assistance) to balance blood flow to the lungs and the rest of the body.

HLHS surgery may involve a series of three surgeries. In some cases, a child with hypoplastic left heart syndrome may need a heart transplant because the case is so complex or because the heart becomes weak after HLHS surgery.

Procedimiento de Norwood

  • The Norwood procedure is done in the first or second week of a baby’s life to allow the right ventricle do the work of the left ventricle — pumping blood to the body — and its normal job of pumping deoxygenated blood returning to the heart back to the lungs.
  • To create this new single-ventricle blood flow, cardiothoracic surgeons create a new aorta to connect to the right ventricle so it can take over the left ventricle’s job pumping oxygen-rich blood to the body.
  • A tube is placed from either the large arteries off the aorta or the right ventricle and connected to the blood vessels supplying the lungs, also known as the pulmonary arteries, so the blood can pick up fresh oxygen.
  • De esta manera, el ventrículo derecho puede bombear sangre tanto a los pulmones como al resto del cuerpo.
  • Once stable, the baby will go home while they grow and build strength for the next procedure in a few months. The family is given a specially equipped iPad and other tools to monitor the baby’s condition and send data at least daily to the team at Norton Children’s Heart Institute. This allows providers to react quickly when a child’s condition changes — sometimes even before the parents realize something is wrong.

Procedimiento de Glenn

  • The Glenn procedure typically is performed when the infant is 4 to 6 months old and is a step toward allowing the right ventricle to perform just the work of the left ventricle, pumping blood to the body.
  • The Glenn procedure allows blood returning from the upper part of the body to go directly into the lungs, bypassing the right ventricle. Los cirujanos cardiotorácicos crean una conexión directa entre la arteria pulmonar y la vena cava superior, el vaso sanguíneo que devuelve la sangre desoxigenada de la parte superior del cuerpo al corazón.
  • The procedure reduces the work of the right ventricle by removing the burden of handling blood returning from the upper part of the body. Blood returning from the lower part of the body still goes through the right ventricle at this point.
La cirugía de SCIH es una serie compleja de procedimientos que requieren habilidad y experiencia.

Procedimiento de Fontan

  • The Fontan procedure typically is performed when the child is 2 to 4 years old to reroute blood from the lower part of the body directly to the lungs and allow the right ventricle to replace the left ventricle.
  • Cardiothoracic surgeons connect the pulmonary artery and the inferior vena cava, diverting oxygen-poor blood from the lower part of the body directly to the lungs.
  • Este procedimiento permite que el resto de la sangre que retorna del cuerpo vaya a los pulmones.
  • Once this procedure is complete, oxygen-rich and oxygen-poor blood will no longer mix in the heart, and the child’s skin will no longer look bluish.
  • Algunos pacientes tendrán una “fenestración” (abertura) que conecta las circulaciones venosa y arterial, lo que permite que se produzca una mezcla periódica.
  • Estas cirugías no curan el SCIH. El niño necesitará visitas de seguimiento periódicas con un cardiólogo pediatra para vigilar el progreso. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist for lifelong care.

Clínica de Fontan

Growing into adulthood with this unique single-ventricle circulatory system can come with complications and risks affecting the liver, kidneys, heart or lungs.

The Norton Children’s Heart Institute Fontan Clinic provides regular follow-up and monitoring to help spot any emerging issue early and treat it sooner, when more options may be available.

The clinic works in conjunction with the Norton Children’s Heart Institute’s adult congenital heart disease program to provide sophisticated care to patients born with heart conditions.

Por qué elegir el Norton Children’s Heart Institute

  • El Norton Children’s Hospital ha sido pionero en cirugía cardiotorácica pediátrica, habiendo realizado el primer trasplante de corazón pediátrico de Kentucky en 1986 y convirtiéndose en el segundo centro en Estados Unidos en realizar un trasplante de corazón infantil.
  • La Junta Americana de Cirugía Torácica ha certificado a nuestros cirujanos cardiotorácicos en cardiocirugía congénita.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • Más de 5,000 niños al año visitan el Norton Children’s Heart Institute para recibir atención cardíaca avanzada.
  • El Norton Children’s Heart Institute tiene consultorios en Kentucky y el sur de Indiana para llevar los cuidados cardíacos pediátricos de alta calidad más cerca de su hogar.
  • La unidad de cuidados intensivos cardíacos (UCIC) Jennifer Lawrence en el Norton Children’s Hospital es la UCIC más grande de Kentucky, equipada con 17 habitaciones privadas y la última tecnología disponible para la atención cardíaca.
La Asociación de Cardiopatías Congénitas en Adultos ha reconocido al Norton Children’s Heart Institute por su experiencia en el tratamiento de cardiopatías congénitas en adultos.

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