International Journal of Cardiology 182 (2015) 4–5

Contents lists available at ScienceDirect

International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard

Letter to the Editor

An original cause of pulmonary hypertension Olivia Domanski a,e,⁎, Emanuela Algeri b, Marie Fertin a,c, Gregory Petit d,e, Nicolas Lamblin a,c,e, Martine Rémy-Jardin b,e, Pascal de Groote a,c a

Service de Cardiologie, Pôle Cardio-Vasculaire et Pulmonaire, Centre Hospitalier Régional et Universitaire de Lille, Lille, France Service de Radiologie et d'Imagerie Cardiaque et Vasculaire, Pôle d'Imagerie et d'Explorations Fonctionnelles, Centre Hospitalier Régional et Universitaire de Lille, Lille, France Inserm U744, Institut Pasteur de Lille, France d Service de Médecine Nucléaire, Pôle d'Imagerie et d'Explorations Fonctionnelles, Centre Hospitalier Régional et Universitaire de Lille, Lille, France e Faculté de Médecine, Université de Lille 2, Lille, France b c

a r t i c l e

i n f o

Article history: Received 5 December 2014 Accepted 25 December 2014 Available online 27 December 2014 Keywords: Pulmonary hypertension Diaphragmatic hernia Atelectasis Catheterization

A 73-year old patient was admitted for shortness of breath (New York Heart Association (NYHA) class III) and fatigue. He is a former smoker and had a history of diabetes mellitus, hypertension, diaphrag-

A

B

matic hernia (known for many years with no history of neonate resuscitation) and prostatectomy for cancer. Physical examination found normal blood pressure (140/80 mm Hg), peripheral oedema and an increase in jugular venous pressure. The ECG demonstrated a normal sinus rhythm at 60 beats/min with a complete right bundle branch block. Arterial blood gas revealed a PaO2 at 67 mm Hg with oxygen saturation at 87%, normal PaCO2 and pH. D-Dimer and troponin levels were in normal values. The chest X-ray showed a left diaphragmatic defect and a clearer left lung in favour of a pulmonary hypoplasia (Fig. 1). The echocardiography found normal left and right ventricular ejection fractions and a dilated right ventricle (RV) at 33 mm; the velocity of the tricuspid regurgitation was 4.45 m/s (with an estimated right ventricle–right atrium gradient of 79 mm Hg) and no argument for an intra-cardiac shunt. The ventilation/perfusion lung scan showed no perfusion of the tracer on the left lower lung confirming the hypoplasia (Fig. 1). The CT-scan displayed a voluminous left diaphragmatic hernia

C

Fig. 1. A. Chest X-ray showing a left diaphragmatic hernia. B. Ventilation/perfusion lung scan showing neither ventilation nor perfusion in the left lower lobe. C. CT scan with reconstruction showing the complete atelectasis of the left lower lobe and the voluminous left diaphragmatic hernia with a protrusion of the spleen and the left colic flexure in the thoracic cage. ⁎ Corresponding author at: Service de cardiologie, Pôle Cardio-Vasculaire et Pulmonaire, Hôpital cardiologique, Boul Prof J Leclercq, CHRU de Lille, 59037 Lille Cedex, France. E-mail address: [email protected] (O. Domanski).

http://dx.doi.org/10.1016/j.ijcard.2014.12.104 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.

O. Domanski et al. / International Journal of Cardiology 182 (2015) 4–5 Table 1 Clinical data, pulmonary function tests and right heart catheterisation results before and after 6 months of sildenafil.

NYHA class 6 MWT (m) Forced vital capacity [l (obs/pr)] Forced expiratory volume 1 s [l (obs/pr)] Total lung capacity [l (obs/pr)] PAP (S/D/M) (mm Hg) PCWP (mm Hg) Cardiac index (ml/min/m2) PVR (dynes/s/cm−5)

Before sildenafil

After sildenafil

III 340 2.45 (72%) 1.96 (76%) 5.24 (84%) 62/13/33 4 2.26 555

II 400 2.92 (87%) 2.12 (84%) 5.87 (94%) 53/15/33 2 2.72 491

D: diastolic; M: mean; MWT: maximal walking test; NYHA: New York Heart Association; PAP: pulmonary arterial pressure; PCWP: mean pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; S: systolic; obs: observed; pr: predicted.

with a protrusion of the spleen and the left colic flexure in the thoracic cage with a complete left lower lobe atelectasis (Fig. 1) associated with a diffuse emphysema, interesting 30 to 40% of the pulmonary volume. A right heart catheterization was performed and showed a precapillary pulmonary hypertension (Table 1). Because of a functional impairment, sildenafil 60 mg per day was started with a significant functional and haemodynamic improvement (Table 1). After 2.5 years, the patient is still improved in NYHA class 2. The congenital diaphragmatic hernia is a rare congenital malformation of the diaphragm that can be complicated by a respiratory failure due to both, pulmonary hypertension and pulmonary hypoplasia [2,3]. Pulmonary hypertension secondary to a diaphragmatic hernia is a well-known disease in infants [4,5]. On the opposite, it has only been

5

described once in adults, however without a confirmatory RHC [1]. In the present case, the pulmonary hypertension is not explained by any right lung malformation, or thoracic deformation, or neonatal resuscitation, or by the emphysema but is probably caused by the left lower lobe atelectasis due to the diaphragmatic hernia known for many years, possibly congenital. The benefit of a surgery is unclear since the expansion of the left lower lobe after several years of atelectasis is uncertain. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References [1] D. Dobarro, B.E. Schreiber, J. Haddock, S. Mandumula, J.G. Coghlan, Bochdalek hernia causing pulmonary hypertension, Circulation 127 (2013) 1837–1839. [2] N. Galiè, M.M. Hoeper, M. Humbert, A. Torbicki, J.L. Vachiery, J.A. Barbera, M. Beghetti, P. Corris, S. Gaine, J.S. Gibbs, M.A. Gomez-Sanchez, G. Jondeau, W. Klepetko, C. Opitz, A. Peacock, L. Rubin, M. Zellweger, G. Simonneau, ESC Committee for Practice Guidelines (CPG), Guidelines for the 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. 30 (2009) 2493–2537. [3] D.B. Badesch, H.C. Champion, M.A. Sanchez, M.M. Hoeper, J.E. Loyd, A. Manes, M. McGoon, R. Naeije, H. Olschewski, R.J. Oudiz, A. Torbicki, Diagnosis and assessment of pulmonary arterial hypertension, J. Am. Coll. Cardiol. 54 (2009) S55–S66. [4] E.B. Rosenzweig, A.C. Widlitz, R.J. Barst, Pulmonary arterial hypertension in children, Pediatr. Pulmonol. 38 (2004) 2–22. [5] D. Bohn, Congenital diaphragmatic hernia, Am. J. Respir. Crit. Care Med. 166 (2002) 911–915.