Ali ORGUN, Niyazi TOKEL, İlkay ERDOĞAN, Birgül VARAN, Murat ÖZKAN, Mehmet Sait AŞLAMACI

Ciddi pulmoner arteryal hipertansiyonlu geniş ventriküler septal defektli olgularının cerrahi sonuçları ve uzun dönem takibi: tek merkez deneyimi

Amaç: Bu retrospektif çalışmada, şiddetli pulmoner arteryal hipertansiyonlu (PAH) geniş ventriküler septal defekt (VSD) nedeniyle ameliyat edilen pediatrik hastalar, klinik özellikleri ve uzun dönem sonuçları açısından ayrıntılı olarak değerlendirilmiş ve güncel ameliyat kriterleri ile yeniden yorumlanmıştır. Gereç ve Yöntem: 1994-2005 yılları arasında PAH’lı geniş VSD nedeni ile ameliyat edilen ve takibi yapılan 24 çocuk hasta çalışmaya dahil edildi. Hastalar pulmoner vasküler resiztans indexine(PVRI) göre iki gruba ayrıldı ve karşılaştırıldı (grup 1:PVRI <6 WU.m² olan olgular, grup 2: PVRI ≥ 6 WU.m² olan olgular). PVRI 6-8 WU.m² olan olgular gri bölgede kabul edildi ve detaylı olarak değerlendirildi. Bulgular: Tanı yaşı 5 ay ile 17 yıl (medyan: 5 yıl) arasındaydı. Ortanca PVRI 5 WU.m² olup 3 -18,4 WU.m² arasında değişmekteydi. PVRI' ye göre 13 hasta grup 1, 11 hasta ise grup 2 olarak kabul edildi. Grup 1'de grup 2'ye göre istatistiksel olarak anlamlı olarak mPAP, PVR/SVR ve son PAP değerleri düşük bulundu. Ameliyat sonrası ortanca takip süresi 6 yıl (1 ay-18 yıl) olup takipte 15 hastada (%62,5) PAH görüldü. Bu 15 hastanın 7'sinde şiddetli PAH gözlenmiş olup bu 7 hastanın tamamı grup 2'deydi (dördünde PVRI ≥8WU.m² ve üçünde PVRI 6-8 WU.m²). Sonuç: Şiddetli PAH gelişmiş geniş VSD'si olan hastalarda, ameliyat için uygun adayları daha doğru bir şekilde belirlemek için ameliyattan önce klinik ve özellikle hemodinamik durumları dikkatlice değerlendirilmelidir.

Anahtar Kelimeler:

Doğuştan kalp hastalığı, pulmoner arteriyal hipertansiyon, ventriküler septal defekt

Surgical outcomes and long-term follow-up of patients with large ventricular septal defects with severe pulmonary arterial hypertension: single-center experience

Purpose: In this retrospective study, pediatric patients who underwent surgery for large ventricular septal defect (VSD) with severe pulmonary arterial hypertension (PAH) were evaluated in detail in terms of clinical characteristics, and long-term results, and reinterpreted with the current surgical criteria. Materials and Methods: Twenty-four patients who underwent surgery for large VSD due to PAH between 1994 and 2005 were included in the study. The patients were divided into two groups according to their pulmonary vascular resistance index (PVRI) and compared (group 1 with PVRI <6 WU.m² and group 2 with PVRI ≥ 6 WU.m²). The patients with PVRI 6-8 WU.m² were accepted as being in the gray zone and were evaluated in detail. Results: Age at diagnosis ranged from 5 months to 17 years (median: 5 years). PVRI ranged between 3 and 18.4 WU.m² (median PVRI was 5 WU.m².) According to the PVRI, 13 patients were accepted as group 1, and 11 patients were accepted as group 2. mPAP, PVR/SVR, and last PAP values were found statistically significantly lower in group 1 than in group 2. The median postoperative follow-up time was 6 years (range, 1 month-18 years). PAH was seen in 15/24 (62.5%) patients. Severe PAH was observed in seven of these 15 patients and all were in group 2 (four had PVRI ≥8WU.m² and three had PVRI 6-8 WU.m²). Conclusion: Patients with large VSD with severe PAH should be carefully evaluated before surgery to more accurately identify suitable candidates.

Keywords:

Congenital heart disease, Pulmonary arterial hypertension, Ventricular septal defect,

PDF

___

Noori NM, Teimouri A. Clinical course in children and adolescents with ventricular septal defect. Asian Cardiovasc Thorac Ann. 2019;27:529-34.
Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest. 2010;137:52-61.
Hu Z, Xie B, Zhai X, Liu J, Gu J, Wang X, et al. Midterm results of “treat and repair” for adults with non-restrictive ventricular septal defect and severe pulmonary hypertension. J Thorac Dis. 2015;7:1165-73.
Hansmann G, Koestenberger M, Alastalo T-P, Apitz C, Austin ED, Bonnet D et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant. 2019;38:879–901.
Kozlik-Feldmann R, Hansmann G, Bonnet D, Schranz D, Apitz C, Michel-Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016;102:42-8.
Xi S-B, Wang S-S, Qian M-Y, Xie Y-M, Li J-J, Zhang Z-W. Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease. Chin Med J. 2019;132:811-18.
Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012;379:537-46.
Alonso-Gonzalez R, Lopez-Guarch CJ, Subirana-Domenech MT, Ruíz JMO, González IO, Cubero JS et al. Pulmonary hypertension and congenital heart disease: an insight from the REHAP National Registry. Int J Cardiol. 2015;184:717-23.
Rosenzweig EB, Krishnan U. Congenital heart disease-associated pulmonary hypertension. Clin Chest Med. 2021;42:9-18.
Aydemir NA, Harmandar B, Karaci AR, Sasmazel A, Bolukcu A, Saritas T et al. Results for surgical closure of isolated ventricular septal defects in patients under one year of age. J Cardiac Surg: Incl Mech Biol Support Heart Lungs. 2013;28:174-79.
Haneda K, Sato N, Togo T, Miura M, Rata M, Mohri H. Late results after correction of ventricular septal defect with severe pulmonary hypertension. Tohoku J Exp Med. 1994;174:41-48.
Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:43-54.
Rosenzweig EB, Abman SH, Adatia I, Beghetti M, Bonnet D, Haworth S et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019;53.
Day RW. Improving guidance for the correctability of congenital cardiovascular shunts with increased pulmonary vascular resistance. Int J Cardiol Congenit Heart Dis. 2021;4:100184.
Roth TS, Aboulhosn JA. Pulmonary hypertension and congenital heart disease. Cardiol Clin. 2016;34:391-400.
Kücükoglu SM, Kaymaz C, Alehan D, Kula S, Akcevin A, Celiker A et al. Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES). Pulm Circ. 2021;11:20458940211024206.
Arvind B, Relan J, Kothari SS. “Treat and repair” strategy for shunt lesions: a critical review. Pulm Circ. 2020;10:2045894020917885.
Akagi S, Kasahara S, Sarashina T, Nakamura K, Ito H. Treat-and-repair strategy is a feasible therapeutic choice in adult patients with severe pulmonary arterial hypertension associated with a ventricular septal defect: case series. Eur Heart J-Case Rep. 2018;2:yty033