Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the
right ventricle
A ventricle is one of two large chambers toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in the uppe ...
of the heart to the
pulmonary artery
A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...
. It is usually first diagnosed in childhood.
Signs and symptoms
Cause
Pulmonic stenosis is usually due to isolated valvular obstruction (
pulmonary valve stenosis
Pulmonary valve stenosis (PVS) is a heart valve disorder. Blood going from the heart to the lungs goes through the pulmonary valve, whose purpose is to prevent blood from flowing back to the heart. In pulmonary valve stenosis this opening is too n ...
), but it may be due to
subvalvular or supravalvular obstruction, such as
infundibular stenosis. It may occur in association with other
congenital heart defect
A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascul ...
s as part of more complicated syndromes (for example,
tetralogy of Fallot
Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:
*pulmonary stenosis, which is narrowing of the exit from the r ...
).
Pathophysiology
When pulmonic stenosis (PS) is present, resistance to blood flow causes right ventricular hypertrophy. If right ventricular failure develops, right atrial pressure will increase, and this may result in a persistent opening of the
foramen ovale, shunting of unoxygenated blood from the right atrium into the left atrium, and systemic
cyanosis. If pulmonary stenosis is severe,
congestive heart failure
Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome, a group of signs and symptoms caused by an impairment of the heart's blood pumping function. Symptoms typically include shortness of breath, excessive fatigue, ...
occurs, and systemic venous engorgement will be noted. An associated defect such as a
patent ductus arteriosus
''Patent ductus arteriosus'' (PDA) is a medical condition in which the ''ductus arteriosus'' fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta, which has ...
partially compensates for the obstruction by shunting blood from the left ventricle to the aorta then back to the pulmonary artery (as a result of the higher pressure in the left ventricle) and back into the lungs.
Treatment
The treatment of choice is
percutaneous balloon valvuloplasty and is done when a resting
peak gradient Peak or The Peak may refer to:
Basic meanings Geology
* Mountain peak
** Pyramidal peak, a mountaintop that has been sculpted by erosion to form a point Mathematics
* Peak hour or rush hour, in traffic congestion
* Peak (geometry), an (''n''-3)-d ...
is seen to be >60mm Hg or a mean >40mm Hg is observed.{{cn, date=November 2021
References
* Ren (Jul. 15, 2009)
Pulmonic Stenosis ''eMedicine Specialties > Cardiology > Valvular Heart Disease''.
* Hockenberry, M. J., & Wilson, D. (2009). Wong's essentials of pediatric nursing. (8 ed., Vol. 1, p. 872). St. Louis, Missouri: Mosby.
Valvular heart disease