Prolonged survival with isolated levocardia and situs inversus

Abstract

Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.

Multiple primary malignancies in a patient with situs ambiguus

“…Most cases (73%) were of single malignancies, in patients with CSI (4-31). There were nine cases of cancer in patients with SA (34)(35)(36)(37)(38)(39)(40)(41), and only one (aside from the present case) reported multiple tumors in the same individual (34). This patient was a 73 years old white female, who had been incidentally diagnosed with abdominal heterotaxia at the age of 59 during a liverspleen scan for tumor staging.…”

Polysplenia Accompanied by Major Cardiovascular Anomalies with Prolonged Survival.

“…However, their case had only minor cardiovascular anomalies (a relatively high position of the tricuspid valve, amonalous pars membranaceaof the ventricular septum and symmetrical conduction system). Vijayakumar and Brandt reported an asymptomatic case of polysplenia in a 73-year-old patient with minor cardiovascular complications (12). In contrast, the present case of polysplenia had major cardiovascular anomalies.…”

Isolated levocardia: Prenatal diagnosis and management

“…Isolated levocardia (IL) is a situs anomaly, characterized by a leftsided heart associated with visceral heterotaxy (Liberthson et al 1973). According to published reports, its estimated incidence is 1 per 22 000 births in the general population and between 0.4 and 1.2% in all patients with congenital heart disease (Campbell & Deuchar 1965;Liberthson et al 1973;Vijayakumar & Brandt 1991). The prognosis depends on the severity of the associated cardiac anomaly, and only 5-13% of IL patients have been reported to survive for longer than 5 years (Harris & Rainey 1965;Annamalai & Ramakrishnan 1967;Liberthson et al 1973).…”