Congenital heart disease: the univentricular heart

By Cristiano Antonino On May 23, 2023

Having a univentricular heart means having a complex congenital heart disease in which one of the two ventricular pumps will not function, either anatomically or functionally

Generally, there will be a lack of separation of the left and right blood circles, and there will be a single pump serving both the systemic circulation and the pulmonary circulation; the two circulations necessary for optimism.

The former will serve the body’s metabolic needs and organ maintenance; the latter will keep the blood oxygenated.

A diagnosis for these pathologies is generally made in the prenatal period with the morphological examination; in the first week after birth, medical evaluations will provide a better understanding of the malformation and identify the best treatment.

The treatment of the univentricular heart will be based on interventions aimed at reducing the symptoms without resolving the causes

This will be followed by an operation to be performed by the age of four, aimed at sending venous blood from the hollow veins to the pulmonary artery.

The forms with non-severe pulmonary stenosis are the best tolerated.

Cyanosis is the predominant symptom.

In the absence of pulmonary stenosis, heart failure will be the predominant symptom.

At first, a pulmonary artery bandage will be performed, decreasing the blood flow to the lungs, or a systemic-pulmonary shunt, which will increase the blood flow to the lungs; subsequently, the second intervention of direct shunting of the superior vena cava into the pulmonary artery will be performed, this is considered the preparatory intervention for correction.

In forms with moderate pulmonary stenosis at birth, this will be the only palliative intervention.

Only one ventricle may be used for correction.

Systemic venous blood will reach the lungs directly for oxygenation and only oxygenated blood for the aorta will pass through the heart.

The risks of such a surgical procedure are high overall, and the results afterwards are contradictory, depending on many variable factors.

Thus, the child, after undergoing surgery, will have systemic and pulmonary circulation, but the lung circulation will not have a dedicated ventricular pump; this is due to the fact that it is possible to survive without a right ventricle.

Thanks to the new interventional procedures, it has been possible to reduce the mortality rate linked to these malformations.

Nowadays, babies born with univentricular hearts, even in their most complex forms, in the vast majority of cases manage to have an almost completely normal examination

For women, born with a univentricular heart, who wish to become mothers, pregnancy may entail specific problems that concern both the intrinsic risk of the pregnancy and the assessment of the possible recurrence of cardiovascular malformations in the foetus.

Depending on the evolution of the malformation of the heart in these patients, it could happen that they might need further interventions, not always surgical; ortho-terminal cardiac transplantation is an example of this.

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