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Dextrocardia is a congenital abnormal position of the heart, with several types, including mirror dextrocardia. Mirror dextrocardia affects all organs in the center of the body, but does not require special treatment. Dextrocardia with abnormal heart is more serious, often requiring surgery, with survival rates depending on the severity of the defects. Treatment options include Rastelli repair and the three-stage Fontan operation. Dextrocardia can also be present in heterotaxy, which involves additional structural abnormalities.
Dextrocardia is a congenital abnormal position of the heart in which, instead of the heart forming in the fetus on the left side, it turns over and forms on the right. There are several types of this condition, also called loop defects. It is often diagnosed on a routine prenatal ultrasound, although not all radiologists detect it, particularly if there are no structural heart abnormalities.
Mirror dextrocardia is a very rare condition, affecting about one in 130,000 people. In this ring defect, not only does the heart flip in the wrong direction and grow out of the wrong side of the chest, but all of the other organs in the center of the body are also reversed. Essentially, an X-ray of someone with this issue looks like a mirror image of the normal positioning of the heart and organs.
This anomaly was first noticed in the 1920s when X-rays revealed this anomalous placement. Fortunately, this type of loop defect does not involve any structural abnormalities of the heart or other organs. In some cases, the cilia — the tiny hair-like structures — in the nose and lungs move in the opposite direction, causing you to be more susceptible to colds or illnesses. Aside from this susceptibility, a person with this condition does not require any special treatment or surgery.
Dextrocardia with abnormal heart is a much more serious condition, usually requiring one or more surgeries to address the structural abnormalities. It is a more common condition than mirror image anomaly, and in most cases, the position of the other organs in the center of the body is not reversed. Outcome and survival rates for this problem depend on the severity of the defects, which generally include a complicated form of transposition of the arteries, called levotransposition, or both arteries arising from the right ventricle, called a double outlet right ventricle.
Large ventricular septal defects are also common in those with this condition. The right and left ventricle can be so similar, unlike the normal heart, that it’s hard to tell which ventricle is which. In many cases, the ventricular septal defect is so large that these chambers are considered to be one, and one or both ventricles may be smaller than usual.
With complicated transposition and single ventricle, two surgical approaches are possible. If the ventricles are large enough, surgeons may choose to perform a Rastelli repair, one and a half ventricles. This surgery builds a tunnel through the heart to reroute blood properly. If possible, the ventricular septal defect can be repaired. Before this treatment, however, the superior vena cava is directed to the smaller pulmonary veins, and blood from the upper body never passes through the right ventricle. This initial procedure, called a Glenn shunt, reduces the heart’s workload and buys time for the baby to grow before the Rastelli’s is done.
Rastelli has an excellent survival rate, once the initial surgery has passed. It is a complicated and often lengthy procedure, with the main drawback being the length of time a child may need to have a heart-lung bypass. A parent should ask a surgeon about their experience and success rate with this or any other surgery. You may need additional surgery in the form of a pulmonic or aortic valve replacement several years after the initial Rastelli.
Levotransposition, small ventricles, and a high degree of pulmonic stenosis in dextrocardia with an abnormal heart generally require the three-stage Fontan operation. In this case, the surgeon performs three surgeries to smooth out the defects. Improved Fontan has led to improved survival rates, and many children born with the condition live into their 30s before needing a heart transplant, the only real solution for single ventricle.
Dextrocardia can also be present in a condition called heterotaxy, which involves not only abnormal positioning of the heart and structural abnormalities, but also the absence of a spleen or the presence of a number of small spleens. Treatment depends on the degree and severity of these structural abnormalities. The lack of a spleen, asplenia, can complicate any operation because the natural resistance to infection is significantly reduced.
