Pulmonary Hypertension in Pregnancy: Critical Care Management

pulmonic stenosis symptoms :: Article Creator

Pulmonic Valve Stenosis

Pulmonic valve stenosis occurs when the pulmonic valve is not able to open normally in systole.

This results in right ventricular pressure overload, right ventricular hypertrophy and eventual right ventricular heart failure.

The vast majority of cases of pulmonic valve stenosis are congenital.

The symptoms include those of right heart failure predominantly dyspnea on exertion.Lower extremity dependant edema can occur and when the legs are elevated at night, the fluid redistributes centrally causing pulmonary edema resulting in orthopnea (dyspnea while laying flat) or paroxysmal nocturnal dyspnea (PND). Hepatic congestion can occur causing right upper quadrant abdominal pain.

Physical Examination

Findings of right heart failure can be seen upon physical examination including elevated jugular venous pressure, hepatojugular reflux and lower extremity edema.

The murmur of pulmonic stenosis is very similar to that of aortic stenosis. It is a midsystolic high-pitched crescendo-decrescendo murmur heard best at the pulmonic listening post and radiating slightly toward the neck, however the murmur of pulmonic stenosis does not radiate as widely as that of aortic stenosis. The murmur of pulmonic stenosis peaks early if the disease is mild and peaks later as the disease progresses.

Also, the murmur of pulmonic stenosis demonstrates increased intensity during inspiration due to the increased venous return to the right heart resulting in greater flow across the pulmonic valve.

While the murmur of aortic stenosis extends up to the A2 component of the S2 heart sound, the murmur of pulmonic stenosis extends through the A2 sound up to the P2 component of the S2 heart sound.

Severe PS results in decreased mobility of the pulmonic valve leaflets and thus a softer P2 sound. Also, as the pulmonic stenosis worsens, the closure of the pulmonic valve is delayed, since more time is required to eject blood through the stenotic valve, resulting in a widely split S2 heart sound that still exhibits inspiratory delay. Note that the murmur of an atrial septal defect is also midsystolic, however it has a fixed split S2.

Diagnosis

Diagnosis is made on echocardiography by visualization of the restricted pulmonic valve leaflets and measuring of the peak gradient across the valve with continuous wave Doppler.

Treatment

Treatment includes pulmonic valvuloplasty.

Mitral Stenosis - Symptoms

Mitral stenosis is often asymptomatic early in disease until the mitral valve area decreases enough to cause a large increase in left atrial pressure. The first symptoms of MS occur on exertion as explained above.

This occurs since the mitral valve area is fixed and the cardiac output is unable to increase enough above resting (a low cardiac reserve is present), and high pressures are transmitted to the pulmonary vasculature since left atrial pressures increase exponentially on exertion. This transmission of pressures results in exertional dyspnea. Fatigue and inability to exercise are also common complaints. Signs of left heart failure such as paroxysmal nocturnal dyspnea and orthopnea can occur. Symptoms of heart failure with concomitant mitral stenosis also occur in disease states that require an increased cardiac output for the same reason such as pregnancy, anemia, sepsis and thyrotoxicosis.

Most patients with moderate to severe mitral stenosis will have some degree left atrial enlargement (LAE) due to the chronic increased LA pressures. This predisposes them to atrial fibrillation. Since people with mitral stenosis rely on atrial contraction for about 20% of their cardiac output and since tachycardia decreases diastolic filling time, the onset of atrial fibrillation with a rapid ventricular rate and loss of atrial contraction results in significant symptoms of low cardiac output and heart failure. These include fatigue, dyspnea, lightheadedness, and even syncope.

In the absence of atrial fibrillation, patients with mitral stenosis still have an increased risk of thrombus formation in their left atrial due to stagnation of blood. This may lead to embolic events including stroke, acute myocardial infarction, acute mesenteric ischemia, or "Blue toe syndrome".

Hemoptysis may occur due to sudden rupture of a bronchial vein. This phenomenon is termed "pulmonary apoplexy". Ortner syndrome may occur when a massively enlarged left atrium compresses the left recurrent laryngeal nerve leading to a hoarse voice. Chest pain from right sided heart strain may occur due to severe venous pulmonary hypertension. Other signs of right heart failure such as right upper quadrant pain (due to hepatic congestion) and peripheral edema may occur.

SUPRAVALVULAR AORTIC STENOSIS WITHOUT WILLIAMS SYNDROME

Supravalvular aortic stenosis (SVAS) is the major cardiac lesion of Williams Syndrome (WS). WS also has features of mental retardation (MR), characteristic facies and variable hypercalcemia in infancy. Most cases of WS probably represent new autosomal dominant (AD) mutations. Isolated SVAS ± peripheral pulmonic stenosis can occur sporadically, however, when reported to occur in more than one family member, it has been assumed by some (McKusick #19405) to be a mild expression of WS. This has major implications for genetic counseling, cardiac evaluation and for the prognosis of the patient and family.

We evaluated 21 members of a 3-generation, 34-member family in which 12 have SVAS documented by ultrasound (US). Four others have SVAS by report of cardiac US done elsewhere. The pedigree was compatible with AD inheritance with high penetrance and variable expression. In 5 tested individuals, the IQ ranged from 102-107; all other members were of normal intelligence by personal observation and interview. No family member had the characteristic facies or other associated findings of WS.

This family study, the largest studied to our knowledge, illustrates that isolated SVAS without WS can be inherited as an AD disorder. Although allelic heterogeneity can not be excluded, this family demonstrates that SVAS and WS are separate clinical entities. Relatives of patients with isolated SVAS should be evaluated for mild signs of disease, and families with SVAS can be reassured that occurrence of MR with WS is unlikely to occur.

Search This Blog

Pulmonary Hypertension