Aurora Cardiovascular Services Case of the Month
ACS Editorial Board
Jasbir S. Sra, MD
Andrew Boyle, MD
Tanvir Bajwa, MD
David C. Kress, MD
Bijoy K. Khandheria, MD
A. Jamil Tajik, MD
Steven Port, MD
Masood Akhtar, MD
Review past cases from 2010.
Complex Congenital Heart Disease Masquerading as Pulmonary Hypertension
Dianne L. Zwicke, MD; Leonard Kleinman, MD; M. Fuad Jan, MBBS, MD; Timothy E. Paterick, MD, JD; David Ponga, RTR, CT, ARRT; A. Jamil Tajik, MD
A 70-year-old woman was referred from St. Vincent Hospital, Green Bay, on April 6 for possible percutaneous closure of a sinus venosus atrial septal defect (ASD). She presented with dyspnea on exertion (DOE) and ankle edema, which had progressed during the preceding year. Her physical function was severely limited (NYHA Class IV). Her past medical history was remarkable only for arthritis and gout. An echocardiographic (Echo) study from the referring institution demonstrated a severely dilated right heart, severe pulmonary hypertension (PHTN) and a normal left heart. The physical exam revealed 3+ edema to the mid thighs, increased p2 component of S2 heart sound with a wide split, right ventricular (RV) heave, grade 2/6 systolic ejection murmur in the pulmonic area, significant jugular venous distension and hepatomegaly.
Transesophageal echocardiography (TEE) was performed on April 12. Again, the large sinus venosus ASD was identified, along with an anomalous pulmonary vein from the right upper lobe of the lung, which emptied into the right atrium (RA) (Figure 1).
Figure 1: Panel A: Apical four-chamber view demonstrating severe enlargement of the right atrium (RA), right ventricle (RV) and the interventricular septum (arrow) bulging into the left ventricle (LV). Panels B and C: Midesophageal view during TEE demonstrating the sinus venosus defect (Panel B, arrow 1), with left-to-right shunt (Panel C, blue color-flow), and the pulmonary vein opening (Panel B, arrow 2 and Panel C, red color-flow) into the defect. Also, note the severely enlarged RA. IAS = interatrial septum; LA = left atrium.
Following the TEE, the patient was taken to the catheterization laboratory where she underwent a right heart catheterization; results are shown in Table 1. Poor hemodynamics made the patient inoperable, as she had a severely dysfunctional RV, RA pressure of 17 mmHg, RV end-diastolic pressure of 25 mmHg, and a pulmonary vascular resistance (PVR) of 10 Wood units when indexed to body mass.
|Table 1. Data Obtained on Initial Catheterization|
|Right atrial||17 mmHg|
|Right ventricular||95/25 mmHg|
|Pulmonary arterial||94/34 (57) mmHg|
|Cardiac output/cardiac input||5.78/2.88 L/min|
|Pulmonary vascular resistance||6.8 WU|
|Oxygen saturation levels|
|Inferior vena cava (IVC)||71%|
|Superior vena cava (SVC)||63%|
|High right atrium||81%|
|Low right atrium||69%|
A chest X-ray demonstrated bilaterally enlarged pulmonary arteries (Figure 2).
Figure 2: Posterior-anterior view of the chest demonstrated enlarged hila. Note the enlarged main pulmonary artery (MPA), right pulmonary artery (RPA) and right atrium (RA). Also, note the pruning of the peripheral vasculature consistent with pulmonary hypertension.
Treatment was initiated with intravenous (IV) prostaglandin (Remodulin) delivered by a Leonard/Hickman double-lumen cuffed tunneled catheter, placed by an interventional radiologist. A follow-up evaluation on July 14 documented marked improvement in all symptoms (decreased DOE, decreased edema, NYHA Class II), with improved Echo findings (Figure 3). However, the right ventricle remained enlarged, with a moderately decreased RV ejection fraction. RV systolic pressure decreased to 64 mmHg.
Figure 3: Panel A: Apical three-chamber view demonstrating severe tricuspid regurgitation (TR) by color Doppler. Panel B: Continuous-wave Doppler through the tricuspid valve demonstrating the maximum velocity of the TR jet with right ventricular systolic pressure of 62 mmHg.
On Aug. 30, a repeat right heart catheterization was performed with the patient on 40 ng/kg/min infusion of Remodulin. The results showed considerable hemodynamic improvement (Table 2). At the time of this procedure, a Swan-Ganz catheter was easily placed into right-upper-lobe and right-middle-lobe anomalous pulmonary veins.
|Table 2. Patient Data After Medical Treatment|
|Right atrial||13 mmHg|
|Right ventricular||63/14 mmHg|
|Pulmonary arterial||61/23 (35) mmHg|
|Cardiac output/cardiac input||5.43/2.34 L/min|
|Pulmonary vascular resistance||2.1 WU|
|Oxygen saturation levels|
|Inferior vena cava (IVC)||71%|
|Superior vena cava (SVC)||68%|
|High right atrium||87%|
|Low right atrium||81%|
A high oxygen saturation value was found in the low RA (RA-IVC junction), raising concern for an additional anomalous pulmonary vein. Therefore, a computed tomographic angiography (CTA) scan of the atria was completed, which showed total anomalous return to the RA from the right lung, each with a separate orifice into the RA, in addition to the large sinus venosus ASD (Figure 4). This newly discovered congenital heart disease required surgery, which was now possible due to the patient’s improved hemodynamics. Remodulin infusion was titrated to a maximum of 47 ng/kg/min preoperatively.
Figure 4: Panel A depicts the sinus venosus ASD in relation to the left and right atria. Panel B depicts the anomalous pulmonary veins leading into the right atrium. RSPV = right superior pulmonary vein; RMPV = right middle pulmonary vein; SVC = superior vena cava.
The patient was electively taken to the operating room for surgical repair of her congenital heart disease on Sept. 20. She tolerated a lengthy and complex surgical procedure very well. The operation consisted of tunneling the anomalous right pulmonary veins into the sinus venosus ASD, utilizing a Dacron patch. The superior vena cava was then enlarged with a pericardial patch. Postbypass TEE revealed no residual shunts and a widely patent SVC.
Her postoperative course was complicated by bradycardia requiring a permanent pacemaker; recurrent pleural effusions requiring bilateral thoracentesis; hypoxia treated at home with 2-liter nasal cannula oxygen; and atrial fibrillation treated with amiodarone. Continuous IV Remodulin therapy was delivered throughout her inpatient stay and after discharge.
The patient suffered a small embolic stroke on Sept. 28. She had complete neurological recovery and is maintained on warfarin (Coumadin) therapy. Starting in late November, she began therapy with oral sildenafil (Revatio), in preparation for cessation of IV therapy. She was slowly weaned off the IV Remodulin infusion and her central line was removed by Jan. 12.
To date, the patient has completed cardiac rehabilitation and a course of lymphedema therapy. Her physical function has improved to NYHA Class I, and she has been weaned off oxygen therapy. She will remain on Revatio for another six months, after which she will be weaned. Although the patient is doing well, she will continue to be followed in our Pulmonary Hypertension Clinic for at least one year to monitor for possible rebound phenomenon.
Pulmonary hypertension has many causes and it is imperative to ascertain the correct etiology. This case is an example of a patient who would have been mismanaged if misdiagnosed, as inadequate preoperative treatment would have resulted in a markedly increased surgical morbidity and mortality rate. Similarly, solely closing the atrial septal defect percutaneously would not have adequately treated her pulmonary hypertension. Careful and methodical evaluation is essential for proper management of patients with complex cardiopulmonary disease, and that is the foundation of care provided by the internationally recognized Pulmonary Hypertension Clinic at Aurora St. Luke’s Medical Center. Cases like the one presented here require a multidisciplinary team approach to achieve an excellent outcome. Our diagnosis and treatment of this patient involved the Pulmonary Hypertension Clinic, interventional cardiologists, a cardiac surgeon trained in repairing congenital heart disease, an interventional radiologist, pulmonary medicine, a cardiac anesthesiologist, an Echo lab with skilled operators, a specialized nursing staff and all support services. Working together, this diverse team converted a patient from needing a (rarely available) heart/lung transplant by surgically repairing her congenital heart disease and resolving her pulmonary hypertension.
Drs. Dianne Zwicke, Leonard Kleinman, Tanvir Bajwa, Bijoy Khandheria, David Olson, Paul Minor and Vladimir Kovacevic contributed to the care of this patient.
Aurora Cardiovascular Services: Phone: (414) 646-2657 or (800) 649-1989.