Abstracto

Total anomalous pulmonary venous connection (TAPVC) and drainage of the inferior vena cava into the left atrium are rare congenital heart diseases. In this case report, we present a newborn patient with the rare combination of these two conditions, diagnosed by chest computed tomography angiography (CTA).

CLINICAL HISTORY

Female patient, on the first day of her life, presented with vomiting, cyanosis and desaturation. Empirical treatment for neonatal sepsis was initiated, without improvement. A chest CTA was performed, which showed the combination of total anomalous drainage of the pulmonary veins and drainage of the inferior vena cava into the left atrium.

IMAGING FINDINGS

A chest CTA showed non-obstructive supracardiac TAPVC, subtype Ia, which occurs when all pulmonary veins converge into a vertical vein, which drains into the left brachiocephalic vein and then into the superior vena cava, which converges into the right atrium (Figures 1 and 2). An ostium secundum interatrial communication was also demonstrated, an essential condition for life in this congenital heart disease (Figure 3). In association with these findings, there was drainage of the inferior vena cava into the left atrium (Figure 4). In the schematic figure it is possible to observe the combination of these changes (Figure 5).

DISCUSSION

Anomalous pulmonary venous drainage is a rare congenital malformation, which accounts for less than 1% of all congenital heart defects [1]. Defects can be partial, when only part of the pulmonary venous circulation has anomalous drainage, or complete, when none of the pulmonary veins drain into the left atrium. Prevalence varies between 0.6 to 1.2 cases for every 10,000 live births [2-4]. The supracardiac subtype is the most common, occurring in approximately 3040% of cases [5], characterized by the final drainage of all pulmonary veins into the superior vena cava. In these cases, atrial septal defect (ASD) is an essential condition. Aberrant drainage of the inferior vena cava (IVC) into the left atrium is also a rare condition and was first described in the literature in 1955 [6]. TAPVC may be associated with several syndromes [5]. There is only one case described in the literature by Lamb et. al. in 1987 of the association between non-obstructive supracardiac TAPVC and anomalous venous return from the IVC to the left atrium [7]. Muir and Prathap, in 1965, described an association between drainage from the IVC to the left atrium and TAPVC, however, with the infracardiac subtype and splenic agenesis [8]. The clinical presentation varies according to the degree of obstruction of the pulmonary vessels. This anatomical change causes volume overload of the right ventricle and pulmonary circulation resulting in congestion and heart failure, as well as generating arterial desaturation due to the mixing of systemic and pulmonary venous blood in the right atrium, through the ASD [9]. Diagnosis is usually made by transthoracic echocardiography. Chest CTA or cardiac magnetic resonance can also be performed for better anatomical characterization [5,10]. Surgical correction is the treatment of choice and should be performed as soon as possible. To our knowledge, this is the second published case in the medical literature describing the association of TAPVC with anomalous drainage from the IVC to the left atrium. In a multidisciplinary meeting, the surgical approach was decided, with redirection of the pulmonary veins to the left atrium and the IVC to the right atrium, as well as closure of the ASD. The patient evolved favorably, being discharged asymptomatic, 18 days after hospital admission.

DIFFERENTIAL DIAGNOSIS

• Subtype Ib supracardiac total pulmonary anomalous venous connection;

• Infracardiac total pulmonary anomalous venous connection;

• Partial anomalous pulmonary venous connection.

TEACHING POINTS

In this case, we learned the importance of each diagnostic imaging method in assessing vascular changes. Despite the correct description of these anatomical alterations, the echocardiogram had limitations in the acoustic window, making it necessary to complement it with CTA. In addition to providing a 3D visualization of the entire heart and the major mediastinal vessels, CTA offers the possibility of selecting the imaging acquisition time after the injection of contrast. The difference in density in Hounsfield Units, represented by the volume of contrast in each of the cardiac compartments, allows us to infer whether there is communication between them, resulting in additional data for a better anatomical vascular characterization.

REFERENCES

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8 - Muir Cs, Prathap K. Co-Existent Total Anomalous Pulmonary Venous Drainage Into Portal Vein, Drainage Of Left-Sided Inferior Vena Cava Into Left Atrium, And Splenic Agenesis. Thorax. 1965 May;20(3):254-60. DOI: 10.1136/thx.20.3.254. PMID: 14292434; PMCID: PMC1018931.

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FIGURES

FIGURE 1: A Chest CTA showed non-obstructive supracardiac TAPVC, subtype Ia: all pulmonary veins (*) converge into a vertical vein (empty arrow), which drains into the left brachiocephalic vein (arrow) and then into the superior vena cava (SVC).

FIGURE 2: A Chest CTA showed that all pulmonary veins (*) are not connected with the left atrium (empty arrowhead). RV = right ventricle. RA = right atrium. Arrowhead = aorta.

FIGURE 3: Transthoracic echocardiography showed interatrial communication due to ostium secundum (empty arrow). RA = right atrium. LA = left atrium. RV = right ventricle. LV = left ventricle.

FIGURE 4: A Chest CTA showed the drainage of the inferior vena cava (IVC) into the left atrium (LA).

FIGURE 5: In the schematic figure it is possible to observe the combination of all the changes: TAPVC and anomalous drainage of the IVC into the left atrium. RA = right atrium. LA = left atrium. RV = right ventricle. LV = left ventricle. AO = aorta. PA = pulmonary artery.

Artículo recibido en miércoles, 13 de noviembre de 2024

Artículo aceptado el sábado, 11 de enero de 2025