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Combined Texas Heart Institute and Texas Childrens Hospital case presentation:

46 year old Woman with Progressive Dyspnea on Exertion
Dr. Rozo J.C., Dr. Springer A., Texas Heart Institute
Dr. Ringewald J., Dr Grifka R., Texas Children's Hospital

Presentation

46-year-old white female c/o progressive dyspnea on exertion. She had a relatively normal childhood growth and development. She was noted to have a murmur 26 years ago. No therapy was sought and the patient had been well until recently. She had a successful pregnancy and delivery in the past without hemodynamic compromise.

Past History

  • Chronic back pain, DM, HTN.
  • Medications: Effexor, progesterone.
  • Allergies: Codeine and Darvon.
  • Surgeries: hysterectomy.
  • Social history: Lives in Louisiana, with her daughter.
  • + Tobacco (1 pack a day)/ ETOH / IVDA.
  • Family History: unremarkable.

Physical Exam

  • VS: BP: 140/70  HR: 74 RR: 20 T: 37.1 Wt: 105 kgs.
  • Gen: AAO x 3, NAD.
  • HEENT: WNL.
  • Neck: Left carotid bruit. thyromegaly, JVD.
  • Heart: normal S1, S2, harsh GIII/VI SEM with an early ejection click, best heard on 2LSB radiated to the left supraclavicular area, not radiating to the back. Click decreased in intensity with inspiration, no diastolic component.
  • Lungs: clear to auscultation bilateral.
  • Abdomen: abdomen: NT/ND, H/S/M.
  • Extremities: no clubbing, cyanosis or edema, 2+ distal pulses.

Heart Sounds

Electrocardiogram

NSR. No evidence right ventricular hypertrophy. Normal right atrium, left ventricular strain pattern.

Chest X-Ray PA and Lateral

Chest examination, normal heart size. The main pulmonary artery is prominent, suggesting pulmonary valve stenosis.

Laboratory Findings

  • CBC: WBC: 6.6, Hb: 13.2 mg/dl, Hct: 35%, Platelets: 268
  • 7/60: Na: 143, K: 4.1, Cl: 105, CO2: 26, BUN: 12, Cr: 0.73, Gluc: 106

Echocardiogram

    mpeg file 1.7 mb

    Click here for pulmonary artery dilatation echo mpeg file (460 KB)

  • Normal left ventricular chamber size. Mild concentric left ventricular hypertrophy. LV function is normal. Overall wall motion is normal. Estimated EF 55-60%. Pulmonic valve not well visualized. The right pulmonary artery is dilated >3.5 cm.
  • PV valve assessment:
  • Peak velocity 345 cm/s.
  • Mean velocity 210 cm/s.
  • Peak gradient 48 mmHg.
  • Mean gradient 25 mmHg.

Cardiac Catheterization

Hemodynamics

Valvuloplasty Procedure

Standard technique for percutaneous balloon pulmonary valvuloplasty (PBPV) was performed over a guide wire from the femoral vein approach, as first described by Kan et.al.

Initially a right heart study was performed using an 8-100 NIH catheter. This confirmed normal intracardiac anatomy and excluded atrial communication. A right ventriculogram in the AP (424 KB) and lateral projection (423 KB) was performed to confirm the diagnosis, to measure the size of the pulmonary valve annulus, and evaluate the main and branch pulmonary arteries. The catheter crossed the pulmonary valve and the peak systolic gradient was measured. To identify infundibular or subvalvular narrowing of the right ventricular outflow tract, a second catheter was placed across the pulmonic valve.

Right ventricular pressure just below the pulmonic valve in the outflow tract was compared to the right ventricular pressure in the apex.

The right ventricular pressure pullback demonstrated a small infundibular gradient.

A 0.035" super stiff exchange gauge wire was placed in the distal artery branch of the right lower lobe and a second super stiff exchange wire was placed in the left lower lobe. XXL 16 mm x 4 cm and XXL 18 mm x 4 cm balloon catheters were inserted through each femoral sheath over the wire, across the pulmonary valve.

The pulmonary valve location on the angiogram was used to center the balloons across the pulmonary valve. The balloon catheter is held in position to prevent forward movement during systole. The balloon is fully inflated until the valve waist is abolished. This is followed by rapid balloon deflation. The inflation procedure was repeated two times. AP view cine (1.46 MB) , LAT view cine (1.31 MB)

The diameter of the 2 balloons was chosen according to the pulmonary valve annulus diameter. The optimal balloon to valve ratio is 1.3 to 1.5. After inflation, the balloon was replaced by a multitrack catheter to evaluate the result of the valvuloplasty procedure. The postvalvuloplasty hemodynamics demonstrated a significant decrease in pulmonary gradient. A post-valvuloplasty right ventriculogram was performed. AP view cine (890 KB) , LAT view cine (1.23 MB)

After the right heart catheterization was completed a left heart catheterization with selective coronary angiograms was performed, with non-obstructive coronary plaques.

Pulmonic Stenosis

Obstruction of right ventricular outflow is valvular in 90% of patients. In the remaining 10%, it is supravalvular or subvalvular.

Supravalvular Pulmonary stenosis

  • Results from the narrowing of the main pulmonary artery, its bifurcation, or its peripheral branches.
  • It often coexists with other congenital cardiac abnormalities:
    Valvular pulmonary stenosis, ASD, VSD, PDA, TOF.
  • It is a common feature of Williams syndrome, which is characterized by infantile hypercalcemia, elfin fascies, mental retardation, in addition to supravalvular and branch pulmonary stenosis.

Subvalvular Pulmonary stenosis

  • Narrowing of the right ventricular infundibulum usually occurs in association with VSD.
     

Valvular Pulmonary stenosis

  • Typically, this is an isolated abnormality, but it may occur in association with atrial septal defect, ventricular septal defect, and hypertrophic subpulmonary stenosis.
  • The valve leaflets are usually thin; all 3 valve cusps are present, and the commissures are fused. During ventricular systole, the valve is dome-shaped with a small central orifice. Among patients with valvular stenosis, 10 to 15 % have dysplastic leaflets.
  • About two-thirds of patients with Noonan's syndrome have pulmonary stenosis due to valve dysplasia.

Degree of stenosis

Mild

  • VA > 1 cm2/m2 BSA.
  • Transvalvular gradient < 40 mmHg.

Moderate

  • VA 0.5-1.0 cm2/m2 BSA.
  • Transvalvular gradient 40-70 mmHg.

Severe

  • VA < 0.5 cm2/m2 BSA.
  • Transvalvular gradient > 70 mmHg, RV pressure > 100 mmHg.

The valve gradient is only helpful with preserved RV systolic function. If the patient has RV failure, a gradient of 35 mmHg may be severe.

Therapy Modalities

Conservative Management

  • Essentially all patients with mild PS can be managed conservatively.
  • Heart failure rarely develops, and most of these individuals need only SBE prophylaxis for surgical and dental procedures.

Surgery

  • Surgical approach has mortality rate of 1.5% to 2%, and significant associated morbidity:
    • Neurologic complications associated with cardiopulmonary bypass, infections, dysrrhythmias, bleeding, transfusions, pain and postpericardiotomy syndrome.
  • Nonfatal complications have been estimated at 12%.

Balloon valvuloplasty

  • Symptomatic and asymptomatic individuals with moderate to severe PS should undergo balloon valvuloplasty.
  • Excellent relief of the pulmonary valve gradient, as well as symptoms, is usually obtained.
  • Although pulmonary valvular stenosis is not uncommon in adults, there are relatively few large series that report this procedure in adults.
  • Antibiotic prophylaxis against SBE should be continued with dental or surgical procedures, even after successful pulmonary valvuloplasty.
  • The first description of pulmonary valvuloplasty was reported in 1982 by Kan, et.al. in children, and in adults by Pepine. He reported successful reduction of the valve gradient in a 59-year-old woman. Balloon valvuloplasty has become the initial therapeutic approach in patients with pulmonic stenosis.

Complications

  • PBPV is generally well tolerated with few major or minor complications.
  • Previously femoral or iliac venous thrombosis has been observed in 3 to 10% of young children and not described in adults. The incidence of venous complications has been greatly reduced by using sheaths in the femoral veins, and lower profile balloons.

Reported Complication Rates Following PBPV

Reported as the major complications by the Valvuloplasty and Angioplasty of Congenital Anomalies Registry (VACA).

  • Death (0.2%).
  • Cardiac perforation (0.1%).
  • Tricuspid insufficiency (0.2%).
  • Other Complications Following PBPV:
    • Septicemia
    • Infundibular obstruction is more common in older patients and responds to treatment with calcium channel blockers or beta-blockers.
    • Significant pulmonary regurgitation is rare and is seen more often after surgical procedures (especially valvectomy).

Infundibular Obstruction: the Suicide Right Ventricle

  • Patients with valvular pulmonary stenosis may have concomitant infundibular narrowing with or without associated right ventricular outflow tract gradients.
  • Beta-blockers may be advisable in patients who have developed significant right ventricular outflow tract gradients following PBPV.
  • Prophylactic use of beta-blockers has also been successfully employed prior to PBPV in the setting of severe infundibular narrowing to prevent the development of dynamic right ventricular outflow obstruction.

Discussion

  • PBPV is a useful and effective treatment in children with congenital pulmonary valve stenosis with few complications. There is a low re-stenosis rate in children. In the literature there are 8 to 10 large series studies in adults.
  • The present case has demonstrated the feasibility, efficacy and safety of this procedure in adults.
  • There were no complications after the procedure.
  • The double balloon technique used in this patient, allows blood to be ejected between the inflated balloons during systole. The double balloon technique causes less ventricular stress, resulting in fewer arrhythmias and better ventricular recovery. Using 2 balloons, smaller balloons are needed (as compared to one large balloon), which require smaller femoral venous sheaths to be inserted, further decreasing vascular trauma. Also, the smaller balloons inflate and deflate more rapidly, further decreasing ventricular stress during balloon inflation.
  • Previous follow up of patients with PBPV have demonstrated sustained improvement. Chen, et. al., reported 53 adolescent or adult patients (age range 13-55 years) who underwent PBPV using the Inoue balloon with excellent short-term and long-term results that were similar to the excellent results in children.
  • In the series by Herrmann, et. al., echocardiographic follow up was obtained with a mean of two years after the procedure. There was a sustained decrease in pulmonary valve gradient.
  • Kaul, et. al., report that post event procedure gradients could resolve spontaneously on follow-up, although assessing the subvalvular gradients correctly after valvuloplasty is often technically difficult. In particular, infundibular stenosis that accompanies chronic severe pulmonary valve stenosis is usually dynamic in nature and was found in two of their patients.

Conclusion

  • Pulmonary valvuloplasty is a well tolerated and effective treatment for pulmonary valve stenosis in adults, with few complications and avoiding the need for surgery.
  • This procedure is the primary treatment of adult patients with pulmonary valve stenosis.
     

Bibliography

Percutaneous balloon valvuloplasty: A new method for treating congenital pulmonary valve stenosis. Kan JW, White RI et al. New England Journal of Medicine 1982:307: 540-542.

Medical progress: congenital heart disease in adults: first of two parts. Brickner M, Hillis Et al. New England Journal of Medicine 2000: 342(4): 256-263.

Second natural history study of congenital heart defects: results of treatment of patients with pulmonary valvar stenosis. Hayes CJ, Gersony WM, Driscoll DJ, et al. Circulation 1993; 87:Suppl I:I-28-I37.

Percutaneous balloon valvuloplasty for congenital pulmonary valve stenosis in adults. Morton J. Kern, Richard Bach. Hemodynamic rounds series II: Pulmonic Balloon Valvuloplasty. Catheterization and Cardiovascular diagnosis 1998, 44:227-234.

Balloon pulmonary valvuloplasty: results of the valvuloplasty and angioplasty of congenital anomalies registry. Stanger P, Cassidy SC el al. Am J. Cardiol 1990;65:775-83.

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