An Unusual Case of Levocardia with Situs Inversus
Authors: Walker Cam , Hankin Mark , Nelsen Sylvia 
Published: June 2021

Introduction

Recently, an unusual case of situs inversus (SI) with levocardia (left‐sided apex of heart) was recognized in a 98‐year‐old female body donor at Oregon Health & Science University (OHSU). A review of the donor medical records gave insight as to her long‐lasting and generally robust health into her 90s. She had three children and lived with no apparent ill effects, aside from a hiatal hernia. Despite having an appendectomy and hysterectomy in life, SI with levocardia went unmentioned in her medical records.

Methods and Results

Additional to the levocardia, superior the diaphragm the OHSU donor presented with a persistent left superior vena cava (SVC), enlarged right atrium, absence of the hepatic segment of the inferior vena cava (IVC), and transposition of the azygos venous system, which emptied in to the SVC. Her right lung had two lobes, as does her left. Inferior to the diaphragm, she had a midline liver with a slightly enlarged left lobe, stomach (with large hiatal hernia) and spleen on the right, complete inversion left to right of the digestive tract (cecum on left), and transposition of the abdominal aorta and IVC.

Discussion

SI with levocardia is extremely rare, with a reported incidence of 1:22,000. It is associated with atrial and ventricular septal defects, a high mortality rates, and is described in the literature as an autopsy finding in extremely young patients (see Abdullah et al. 2015; Mittal et al. 1994; Campbell & Deuchar 1967; Padmavati & Gupta 1962). The condition is associated with congenital heart disease, asplenia or polysplenia, and has a 5‐year survival rate of 5–13% (Abdullah et al. 2015; Vijayakumar and Brandt 1991). In a study of 19 patients presenting with partial or complete SI from 1973 to 2001, only one has presented with levocardia, the remainder with dextrocardia (Fulcher & Turner 2002). Congenital heart disease incidences with SI are low, with one study noting 3 to 5% (Fulcher & Turner 2002), while another suggested 0.4 to 1.2% of patients with congenital heart disease also had SI (Vijayakumar & Brandt 1991). Only Vijayakumar and Brandt (1991) report long term survival (patient aged 73) of an individual with both levocardia and SI. Conversely, SI with dextrocardia (right‐sided apex of heart) is often associated with a normally functioning heart, particularly with normally arranged great vessels, as is often the case (Mittal et al. 1994; du Plessis & Loukas 2016; Spratt 2016).

Conclusion

This combination of anatomical anomalies appears to be extremely rare, and numbers of individuals are infrequently reported in clinical literature. Only one older survey article (Campbell and Deuchar 1967) has attempted to determine overall rate of occurrence in the general population. When comparing the incidence of SI (0.02 of general population) with other categories, including symmetry of liver and absent IVC (both present in the OHSU donor), the authors suggest that 1% or even less of the persons with SI in the general population have this combination of anatomical variation (Campbell and Deuchar 1967). In this poster presentation, we will present illustrations of the donor's anatomical arrangement of thoracic and abdominal viscera, some implications for imaging, and briefly explore possible embryological origins of these anomalies along with their heritability.

This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.