Totally anomalous pulmonary venous drainage - supracardiac type: ultrasound assessment of anatomically determined stenosis of the vertical vein collecting pulmonary venous return and Ultrasound diagnosis of pulmonary sling with proximal stenosis of left pulmonary artery and patent arterial duct.

Abstract

Totally anomalous pulmonary venous drainage was for the first time described on the basis of a post-mortem examination in 1798(1). In the 21st century such pathologies are diagnosed in a living patient. The echocardiographic detection and diagnosis of totally anomalous pulmonary venous drainage (TAPVD) is a real challenge for every cardiologist. This defect is encountered rarely and it is difficult to be precisely evaluated in young patients. The diagnosis usually requires the application of additional imaging techniques such as 3D CT. With great interest I have read the precise description, actually an echocardiographer's “instruction,” on how to move the ultrasound beam step by step as well as how to interpret the registered 2D or color Doppler images in order to trace the supracardiac type of anomalous pulmonary venous drainage(2). Such a clear and precise description is a genuine pearl in our literature. Particular attention should be drawn to additional (apart from routine) projections and application sites of the ultrasound transducer, i.e. parasternal and high parasternal ones. They are valuable since they extend our sonographic “window” and the classical echocardiographic examinations in parasternal projection belong to the worst tolerated ones by young patients examined both in an outpatient clinic and in the ICU. I wish to congratulate the authors on their beautiful photographic documentation. Seizing the opportunity to share my own experience as an academic teacher with the Authors of the commented paper and the Readers, I wish to remind that the discussed defect appears in the embryonic period and thus, it is present during ultrasound examinations both in 13th–14th and 18th–20th weeks of gestation as well as later. This pathology may be diagnosed in the fetal cardiac center(3–5). So as to transfer this information to the recipients in the clearest way possible (and both medical workers such as obstetricians, neonatologists or pediatric cardiologists as well as parents should be familiar with it), in the center in Łodź, we try to attach a graphic presentation of the defect to each examination result. Anomalous pulmonary venous drainage may occur as the supracardiac type, as discussed in the commented article, but there are also intracardiac and infracardiac types. Each of them may be identified in fetuses. In 2004–2013 in the Polish National Registry of Fetal Cardiac Pathology (www.orpkp.pl), we have registered 19 such cases out of 5,009 fetuses with cardiac defects – they constituted 0.3%. Children with such anomalies are usually referred for a scheduled cardiac surgery. However, in the case of stenosis of the pulmonary veins, the patient's condition may worsen rapidly which might result in their death before relevant treatment is implemented. Pulmonary vein stenosis in a fetus has so far been a lethal cardiac defect(4, 6). Nevertheless, a good prenatal diagnosis and efficient transfer of the neonate to the cardiac catheterisation laboratory might in the future create a possibility to treat the narrowing by means of a stent and to perform further surgical corrections. The second interesting paper(5) is, in my opinion, a real rarity. No similar prenatal diagnoses are mentioned in the Polish National Registry of Fetal Cardiac Pathology and in the MEDLINE database, only several such cases may be found(7–9). It is worth reminding that sling may be present in fetuses with asymmetrically developed lungs. To sum up, the Authors of the commented papers have served a real spiritual feast for the Readers of “Journal of Ultrasonography” who deal with cardiology and echocardiography.