Overview
Criss-cross heart is an extremely rare congenital heart defect, meaning it is present from birth. In a normal heart, the right and left sides are arranged in a predictable way, with blood flowing in an organized path from the body to the lungs and back. In criss-cross heart, the heart is twisted on its own axis, causing the paths of blood flow from the two main pumping chambers (the ventricles) to cross over each other like an X — hence the name. This unusual twisting changes how the heart sits inside the chest and how its chambers connect to the major blood vessels. Because of this abnormal arrangement, criss-cross heart almost always occurs alongside other serious heart defects. These commonly include a hole between the two pumping chambers (ventricular septal defect), abnormal connections between the heart chambers and the large arteries (such as transposition of the great arteries), and narrowing of the pulmonary valve or artery that carries blood to the lungs. Together, these problems mean the heart cannot pump blood efficiently, leading to low oxygen levels in the blood. Babies born with criss-cross heart typically show signs of serious heart trouble very early in life, including bluish skin color (cyanosis), rapid breathing, and difficulty feeding. Treatment requires surgery, often in stages, and the specific approach depends on which additional heart defects are present. Because of its rarity and complexity, this condition is best managed at specialized pediatric heart centers with experienced surgical teams.
Also known as:
Key symptoms:
Bluish or purple tint to the skin, lips, or fingernails (cyanosis) due to low oxygenRapid or labored breathingDifficulty feeding or tiring quickly during feedingPoor weight gain in infancyHeart murmur heard by a doctorSwelling in the legs, abdomen, or around the eyesExtreme fatigue or low energyFainting or loss of consciousness in older childrenReduced ability to exercise or play without becoming breathless
Clinical phenotype terms (13)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Criss-cross heart.
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Specialists
View all specialists →No specialists are currently listed for Criss-cross heart.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Criss-cross heart.
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What specific heart defects does my child have in addition to the criss-cross arrangement, and how do they affect the treatment plan?,Is my child a candidate for a full two-ventricle repair, or will they need staged palliative surgery like the Fontan procedure?,What are the risks and expected outcomes of the recommended surgery at this center?,How often will my child need follow-up appointments and what tests will be done at each visit?,Are there any activity restrictions my child should follow at home or at school?,What warning signs should prompt me to call you or go to the emergency room immediately?,Should we seek a second opinion at another specialized pediatric heart center?
Common questions about Criss-cross heart
What is Criss-cross heart?
Criss-cross heart is an extremely rare congenital heart defect, meaning it is present from birth. In a normal heart, the right and left sides are arranged in a predictable way, with blood flowing in an organized path from the body to the lungs and back. In criss-cross heart, the heart is twisted on its own axis, causing the paths of blood flow from the two main pumping chambers (the ventricles) to cross over each other like an X — hence the name. This unusual twisting changes how the heart sits inside the chest and how its chambers connect to the major blood vessels. Because of this abnormal
How is Criss-cross heart inherited?
Criss-cross heart follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Criss-cross heart typically begin?
Typical onset of Criss-cross heart is neonatal. Age of onset can vary across affected individuals.