Background
Ventricular septal defect (VSD) is the most common congenital heart anomaly. The most frequent location is the membranous septum. The volume and direction of the shunt depends on the size of the VSD, pulmonary vascular resistance (PVR) and the right and left ventricular function. Adult patients with VSD are often after surgical treatment in childhood. Untreated patients may have small VSD with left-right shunt, without LV overload and pulmonary hypertension (PH); VSD with left-right shunt and PH or VSD with Eisenmenger syndrome.
Case Presentation
A 66-year-old woman with perimembranous ventricular septal defect (VSD) was admitted to our hospital with progressive dyspnea on exertion for cardiac catheterization and qualification for further treatment. She had a history of chronic heart failure, chronic obstructive pulmonary disease, permanent atrial fibrillation, hypertension, diabetes mellitus t. 2 treated by insulin, hypothyroidism, depression, obesity and mild aortic stenosis. In 2005 she had a cardiac pacemaker implanted due to tachycardia-bradycardia syndrome.
The patient was admitted to our hospital with symptoms of heart failure- class III according to the New York Heart Association (NYHA). Physical examination showed heart rate 85 bpm, blood pressure 110/70 mmHg, systolic murmur, vesicular breath sounds and slight pedal oedema. The blood saturation was 90-92% in upright position and decreased to 85% after exercise and in supine position. Her height was 150 cm, weight 107 kg, calculated body mass index (BMI) was 48 kg/m². The ECG showed atrial fibrillation with paced ventricular rate 85 bpm and QRS width of 150 ms. According to pacemaker data almost 95% of QRS complexes were paced. The laboratory tests revealed raised red blood cells 5.25 10³/µl, hemoglobin 16.4 g/dl, hematocrit 49.0% and elevated TSH 6,36 µIU/ml [0,27-4,2]. White blood cells, platelets, FT3, FT4, creatinine, hsCRP and ALT were within the limits of normal. Computed tomography excluded pulmonary thromboembolism. Her spirometry showed restrictive ventilation pattern. Echocardiography showed enlargement of the right ventricle (RV) and both atria, impairment of the left ventricle (LV) with ejection fraction (EF) 35%, mild aortic stenosis with pressure gradient 20/8 mmHg, mild mitral and tricuspid valve regurgitation, restrictive perimembranous VSD with left-right shunt and pressure gradient 80 mmHg, right ventricular systolic pressure 55 mmHg. Coronary angiography showed normal coronary arteries. Right heart catheterization revealed VSD with left-right shunt (Qp:Qs= 1,53:1), non- reversible pulmonary hypertension (mean pulmonary artery pressure 49 mmHg, after NO inhalation 51 mmHg, vascular pulmonary resistance 416 ARU, after NO 382 ARU), low aortic blood saturation (88.9%, after NO 88.3%) and high pulmonary capillary wedge pressure (PCWP) ( 26 mmHg, after NO 27 mmHg).
Discussion and current guidelines
According to current guidelines VSD should be treated surgically in symptomatic patients without severe pulmonary vascular disease (IC) and in asymptomatic patients with LV volume overload caused by VSD (IC).[1] The surgical closure of VSD should be considered when there is a history of infective endocarditis (IIaC) or in the presence of aortic regurgitation caused by aortic valve cusp prolapse associated with VSD (IIaC). In patients with VSD and PAH the surgery should be considered when there is still left-right shunt with Qp:Qs>1.5 and PAP or PVR are under 2/3 of systemic values.[1] The VSD should not be corrected in Eisenmenger’s syndrome or when there is blood desaturation during exercise (IIIC).[1] In this case we did not decide to close VSD because of the high PCWP and high risk for surgery. We did not qualify this patient for specific treatment of PH, either because of concomitant lung disease and heart failure that seem to be the main etiology of PH. In PH caused by lung diseases and left heart disease the use of PAH specific drugs is not indicated.[2] There are reports suggesting that upgrading from single- to biventricular pacing in patients with continuous right ventricular pacing can reduce dyssynchrony, improve ventricular function and diminish symptoms. [3,4] Because of the wide QSR during right-ventricular stimulation and progressive symptoms this female patient was selected for cardiac resynchronization therapy (CRT).
References
1. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010 Dec;31(23):2915-57
2. Galiè N, Hoeper MM, Humbert M, et al. Guidelines for he diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009 Oct;30(20):2493-537.
3. Vatankulu MA, Goktekin O, Kaya MG, et al. Effect of long-term resynchronization therapy on left ventricular remodeling in pacemaker patients upgraded to biventricular devices. Am J Cardiol 2009; 103:1280 –1284.
4. Paparella G, Sciarra L, Capulzini L, et al. Long-term effects of upgrading to biventricular pacing: differences with cardiac resynchronization therapy as primary indication. Pacing Clin Electrophysiol 2010;33:841 –849.
Experts’ comments:
1. Anna Fijałkowska: The patient does not qualify for treatment of PH due to high PCWP.
2. Olga Trojnarska: Dysfunction of the left ventricle is the key reason for deterioration of
the clinical state, thus closure of the moderate VSD will not cause the expected improvement. Increased pulmonary pressure does not depend on the shunt, but originates mainly from the venous circulation. Therefore, pharmacological therapy should be administered.
3. Janusz Skalski, Zbigniew Gąsior: Percutaneus closure of VSD should be taken into account.
4 Grzegorz Kopeć, Monika Komar: There is a low probability that VSD is the main reason for symptoms in this patient. Pulmonary hypertension results in this case not only from the shunt but also from concomitant diseases such as left ventricular dysfunction and pulmonary diseases.
5. Piotr Podolec: The patient should be considered for resynchronization therapy, additionally depressive symptoms in this patient should be further investigated.
6. Anna Prokop-Staszecka: Further diagnosis of Sleep Apnea Syndrome (SAS) should be performed because that could be the reason for blood desaturation at night and in the supine position. Surgical treatment of obesity could also be considered but the high overall risk in this patient will probably discourage surgeons from this procedure.
Conclusions:
The patient described in this report has PH of mixed etiology (due to congenital heart disease, lung disease and left heart disease). She has also many concomitant diseases that limit treatment options. There is a low probability that VSD is the main reason for symptoms in this person. Dyssynchrony that occurs during right ventricular pacing deteriorates LV function, therefore to cancel that effect and reduce the symptoms she was selected for CRT. She has been disqualified from specific PH treatment because there is no data that it could be helpful in a patient with PH due to VSD, left ventricular dysfunction and COPD. The important disease in this woman is obesity but invasive treatment is not recommendable because of high surgical risk. This patient should also be investigated for Sleep Apnea Syndrome (SAS). She has been informed about the importance of weight lost and in 4 weeks she lost 5 kg. The patient continues with optimal pharmacological treatment.
She should be classified in group I.3. in the classification of rare cardiovascular diseases.
Authors:
Joanna Luszczak1, Lidia Tomkiewicz- Pajak1, Maria Olszowska1, Urszula Sutor1, Piotr Podolec1, Anna Prokop-Staszecka 6
Experts:
Anna Fijałkowska2, Olga Trojnarska3,Janusz Skalski4, Zbigniew Gąsior5, Paweł Rubiś1, Grzegorz Kopeć1, Piotr Podolec1
1 Department of Cardiac and Vascular Diseases, John Paul II Hospital, Kraków, Poland
2 Department of Chest Medicine, Institute of Tuberculosis and Lung Diseases, Warszawa, Poland
3 1st Department of Cardiology, University of Medical Sciences, Poznań, Poland
4 Department of Pediatric Cardiology, Polish–American Children’s Hospital, Kraków, Poland
5 Department of Cardiology, Medical University of Silesia, Katowice, Poland.
6 1st Department of Lung Diseases, John Paul II Hospital, Kraków, Poland
