In this case, a multi-factorial heart failure was considered. The first impression from the radiological pattern was consistent with predominant pulmonary left-side edema related to heart failure. Finally, a radiological hypertransluced pattern on HRCT in the opposite lung was seen to be related to a diminished vascularity and hyperinflation pulmonary parenchyma, characteristically associated with SJMS. The relevant polycythemia was most probably secondary to cyanotic congenital heart disease associated with the newly diagnosed PDA with minimal right-to-left shunt. A PDA rarely closes spontaneously after infancy [
4] and small patent ductus arteriosus cause no symptoms, as it seems in the present case. It is not clear which factor most contributed to the actual clinical picture presented by the patient: the PDA with a minimal inverted shunt; the parenchyma abnormalities resulting in differential pulmonary blood flow due to SJMS; or even the respiratory obstructive lung disease component seen on PTF. A cardiac catheterization would eventually be necessary but, at this point, she declined further evaluation. Nevertheless, the lack of normalization in arterial hypoxia in the presence of oxygen supplementation most probably shows a non-invasive confirmation of the major contribution of inverted shunt PDA which may explain the development of pulmonary arterial hypertension and consequent heart failure. The normal size right cardiac chambers shown in TTE should be better characterized with trans-esophageal echocardiography which was also declined by the patient.
SJMS is considered to be a relatively uncommon and complex disease characterized by unilateral hyperlucency of a part of or the entire lung which was first described in 1953 by Swyer and James [
3]. It is presently considered to be an acquired disease secondary to viral bronchiolitis and pneumonitis in childhood etiological associated [
1] with
Paramyxovirus morbillivirus,
Bordetella pertussis,
Mycobacterium tuberculosis,
Mycoplasma pneumoniae, influenza A and adenovirus types 3, 7 and 21. Our patient suffered recurrent episodes of pulmonary infections in her childhood. Clinically, patients usually present productive cough, shortness of breath and dyspnea on exertion, sometimes with haemoptysis. Some patients, who have little or no associated sequelae bronchiectasis, have minor symptoms or are asymptomatic and may, therefore, not be diagnosed until they are adults. In our case, the final SJMS diagnosis was reached during a new onset heart failure workup performed when the patent was 53. SJMS diagnosis is based on the radiological pattern such as [
5] unilateral or lobar pulmonary hyperlucency associated with an air trapping lung during expiration ultimately resembling a mosaic pattern. The affected lung parenchyma shows a variable degree of destruction and bronchiectasis could be associated. Those aspects could better explain the obstructive-restrictive respiratory pattern typically seen on PTF and also reported in our patient. In addition, pathological pulmonary artery seen on angio-CT has, typically, a decreased caliber and, consequently, the lung blood flow is reduced. Finally, another characteristic described is that the pathological perfusion changes in the diseased lung sections and the occasionally bizarre hyperinflation ultimately result in the compression of healthy lung areas resulting in atelectasis [
6]. For this reason, the diagnosis of this syndrome is better established with HRCT on inspiration and expiration complemented with an angio-CT. A ventilation-perfusion lung scanning [
7] could also be performed but false-positives could appear in the presence of any disorder involving distal airway obstruction (such as, bronchiolitis obliterans, asthma or congenital lobar emphysema). SJMS treatment includes the early control of lung infections as well as influenza and pneumococcal vaccinations [
2,
5]. Resection of the affected lung was successful [
2]. No specific morbid-mortality studies with SJMS have been done.