Ventricular Hypertrophy due to Hypoplastic Coarctation of the Aorta

Congenital Defect Heart Defect of the Aorta

Coarctation of the aorta and a related hypoplastic condition (small aorta) are congenital diseases, meaning the individual is born with the defect. It is loosely defined as a narrowing (coarctation) or a smaller, less developed form (hypoplasty) of the aorta. The aorta is the blood vessel that carries oxygen rich blood from the heart to the other arteries that distribute the blood to other organs in the body. The aorta forms an arch from the back of the heart curving forwards towards the abdomen. At the top of the curve there are feeder arteries to the head and arms. The narrowing most commonly occurs in the segment just past this point, in a region commonly referred to as the juxtaductal region of the aorta.

The narrowing originates during the patient’s development as a fetus. It is theorized to develop as a result of excess ductal tissue which encroaches into the aorta, causing it to narrow.

Symptoms

The narrowing aorta results in the heart, specifically the left ventricle, having to work harder to force the blood through the narrowed blood vessel. This exertion creates a higher blood pressure which helps to force the blood through the narrower aortic space. However, this exertion taxes the heart and, depending on the severity of the constriction, the ventricle maybe unable to deliver enough pressure resulting in congestive heart failure. In some cases, the ventricle atrophies, which creates other complications.

In newborns, the baby is diagnosed with a narrowed aorta by the following symptoms: the newborn will most likely have a heart murmur, upper limb hypertension, and lowered femoral pulses. The physician will order echocardiography and magnetic resonance imaging to confirm the diagnosis.

If the condition is not diagnosed at the newborn stage, the physiological symptoms continue undetected. Eventually the patient may have exhibit the symptoms of heart failure. The older the patient more symptoms become apparent. They start struggling with tolerance for exercises; their legs tire easily and became cold at the extremities. In extreme cases the patient may experience cardiac issues such as arrhythmia and left ventricular hypertrophy.

Treatment

If the condition is detected in infancy then the newborn is put on an intravenous medication called prostaglandin which helps open the narrowed space. This treatment is only temporary and is employed to keep the patient’s blood flowing prior to surgery.

Surgery is performed on a case by case basis, but is usually imperative. Surgeons will commonly resect the artery, removing the affected section. Another surgical procedure used to open the narrowed section uses a patch (subclavin flap aortoplasty).

An alternative to surgery is a procedure that utilizes a stent. The stent is inserted once the narrowed space is dilated by a balloon.

Whichever treatment a patient follows, long-term follow up treatment is important. This enables the physician to track possible side effects that can result from the procedures. These side effects may include hypertension and restenosis (renarrowing of the artery).

References

  1. Eric Rosenthal, Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart. 2005 November; 91(11): 1495–1502 Article