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(Chest. 2003;124:2068-2073.)
© 2003 American College of Chest Physicians

Lipomatous Hypertrophy of the Interatrial Septum*

A Prospective Study of Incidence, Imaging Findings, and Clinical Symptoms

Christoph M. Heyer, MD; Thomas Kagel, MD; Stefan P. Lemburg, MD; Torsten T. Bauer, MD and Volkmar Nicolas, MD

* From the Institute of Radiology and Nuclear Medicine (Drs. Heyer, Kagel, Lemburg, and Nicolas), and the Department of Pneumology, Allergology, and Sleep Medicine (Dr. Bauer), BG Kliniken Bergmannsheil, Ruhr-University of Bochum, Bochum, Germany.

Correspondence to: Christoph Malte Heyer, MD, Institute of Radiology and Nuclear Medicine, BG Kliniken Bergmannsheil, Ruhr-University of Bochum, Bürkle-de-la-Camp Platz 1, D-44789 Bochum, Germany; e-mail: christoph.m.heyer{at}ruhr-uni-bochum.de


   Abstract
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 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
Background: Lipomatous hypertrophy of the interatrial septum (LHIS) is a benign disorder characterized by fat accumulation in the interatrial septum. It typically occurs in elderly, obese patients and may cause arrhythmia.

Objectives: The purpose of this study was to determine the imaging features of this cardiac pathology using multislice CT (MSCT), and its relationship with clinical findings.

Materials and methods: A total of 1,292 consecutive patients who underwent MSCT of the thorax from September 2001 to August 2002 were prospectively studied. Beside the analysis of patient records, the amount of fat in the interatrial septum, and its size and shape were determined.

Results: Among the 1,292 patients investigated, we found 28 (2.2%) with LHIS. The mean age of affected patients was 72.2 years. MSCT studies revealed a mass of fat attenuation with sharp margins and sparing of the fossa ovalis, resulting in a dumbbell shape in all patients. The median thickness of the interatrial septum was 32 mm (range, 20 to 62 mm), and the median craniocaudal extend was 62 mm (range, 51 to 89 mm). Twenty-one patients (75.0%) showed increased epicardial fat, 18 patients (64.3%) had significant pulmonary emphysema, and 13 of 21 patients (61.9%) showed ECG abnormalities. Three patients underwent functional cardiac MRI studies. In one patient, hemodynamic obstruction by LHIS was shown.

Conclusions: MSCT scanning is a useful method to diagnose LHIS, a cardiac condition that in our series reached a 2.2% incidence. The lesion shows characteristic features, thus differentiating it from other cardiac tumors. Advanced age, obesity, pulmonary emphysema, and atrial arrhythmias are common additional findings in patients with LHIS.

Key Words: cardiac tumors • CT • lipomatous hypertrophy of the interatrial septum • MRI


   Introduction
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 Abstract
 Introduction
Materials and Methods
 Results
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Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare entity that is characterized by the excessive deposition of fat in the interatrial septum and a thickness of > 2 cm.1 2 Former case reports indicate that LHIS usually occurs in elderly, obese patients.1 2 3 Although in most cases LHIS is not clinically apparent, it may cause cardiac arrhythmias. The appearance of LHIS on echocardiograms is well-known.4 5 6 7 A few reports4 6 8 9 10 11 12 13 have described the characteristics of LHIS on CT scans and MRI.

We prospectively analyzed all CT studies of the thorax that were performed between August 2001 and September 2002 in our institution. In addition, three patients with LHIS underwent cardiac MRI evaluation. The typical imaging features of LHIS and the clinical findings in affected patients are described.


   Materials and Methods
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 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
From August 2001 to September 2002, a total of 1,292 patients underwent a contrast-enhanced thoracic CT scan at our institution. All examinations were done on a multislice-CT (MSCT) scanner (SOMATOM Volume Zoom; Siemens; Erlangen, Germany) using a breathhold technique, and identical technical parameters (ie, caudocranial data acquisition, collimation of 4 x 1.0 mm or 4 x 2.5 mm, and slice thickness of 3 or 6 mm). Eighty milliliters contrast agent (Solutrast 300; Byk Gulden; Konstanz, Germany) were administered at a rate of 2.5 mL/s. All CT images were analyzed by two experienced radiologists. LHIS was defined as the presence of a nonenhancing fatty mass within the cardiac interatrial septum and a thickness of >= 20 mm.1 2 In every affected patient, the maximum craniocaudal extend and the maximum thickness of the interatrial septum were measured using three-dimensional (3-D) reconstructions of the initial data set. In addition, the shape of the interatrial mass on cross-sectional images was recorded. An analysis of axial CT scans displayed in the lung window was performed in every patient, and pulmonary emphysema was recorded if present.

An MRI scan of the heart was performed in three patients in whom LHIS was diagnosed by CT scan. The MRI scan was performed with a 1.5-T MRI unit (Magnetom Symphony QUANTUM; Siemens) [maximum gradient, 30 mT/m; slew rate, 125 T/m/s]. Images were obtained with ECG-triggering and breathhold technique. A four-channel, phased-array body coil was used. After acquiring T1-weighted turbo spin echo images of the heart in its orthogonal axes, true fast imaging with steady-state precession cine-sequences, and T2-weighted DB-half-Fourier single-shot turbo spin echo images were obtained.

Body mass index (BMI) was calculated for every patient using the following formula: body weight (kg)/body height (m)2. A BMI of > 30 was documented in every affected patient. ECGs were reviewed when available, and abnormalities were recorded if present. Coronary artery disease was diagnosed in patients having a history of myocardial infarction and/or pathologic findings in a former coronary angiogram.


   Results
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 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
In the 1,292 patients investigated, we found 28 (2.2%; 16 women and 12 men) with LHIS. Clinical findings in our patient population are shown in Table 1 .


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  		Table 1.. Characteristics of Patients With LHIS*

 
The mean age of affected patients was 70.2 years (range, 57 to 87 years). Twelve of 28 patients (42.9%) showed a BMI of > 30. In 13 of 21 patients (61.9%), ECG rhythm abnormalities were present, and 8 patients showed atrial arrhythmias (atrial fibrillation, 5 patients; atrial premature complexes, 2 patients; and atrial tachycardia, 1 patient).

CT studies in affected patients revealed a homogenous mass of fat attenuation with sharp margins. No patient showed significant contrast enhancement of the mass. Sparing of the fossa ovalis and a prominent constriction of the central septum with resulting dumbbell shape of the lesion were found on transaxial images in 26 patients (92.9%) [Fig 1 ]. The dumbbell shape was documented in all patients after the acquisition of 3-D reconstructions (Fig 2 ). The median thickness of the interatrial septum was 35 mm (range, 20 to 62 mm), and the median craniocaudal extend was 62 mm (range, 51 to 79 mm). In 24 patients (85.7%), LHIS ranged from the level of the coronary sinus to the root of the aorta. Three patients showed a lesion that ranged up to the roof of the atria. Twenty-three of 28 patients (82.1%) showed a finger-like projection of fat along the coronary sinus (Fig 3 ), whereas a tapering of fat along the right lateral atrial wall was seen in 19 of 28 patients (67.9%) [Fig 4 ]. In 21 patients (75.0%), increased epicardial and/or mediastinal fat was seen. Eighteen patients (64.3%) had significant pulmonary emphysema (Fig 5 ), and 12 patients of this group received systemic (oral) steroids. No patient had received or was receiving long-term parenteral nutrition. Three patients underwent functional cardiac MRI. In one patient, the circumferential contact between the mass and the entire superior vena cava with slight hemodynamic flow obstruction was shown. Apart from those visible on CT scans, no additional abnormalities were detected on MRI images. None of the patients underwent bioptic confirmation of the intracardiac mass or surgical intervention.



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  		Figure 1.. MRI (top and middle) and CT scan (bottom) of patient 9. The typical dumbbell shape of LHIS with sparing of the fossa ovalis (black arrows) is visible on all transaxial images.

 


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  		Figure 2.. CT scan of patient 6. Top: presence of large LHIS (black arrows) without typical sparing of the fossa ovalis on transaxial images. Bottom: the dumbbell shape (black arrows) is visible in coronal reconstruction.

 


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  		Figure 3.. CT scan of patient 23. LHIS with a finger-like projection of fat along the coronary sinus (black arrows). Also note the increased epicardial fat (white arrows).

 


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  		Figure 4.. CT scan of patient 17. Large LHIS extending anteriorly to the lateral wall of the aorta and tapering the wall of the right atrium (black arrows).

 


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  		Figure 5.. CT scan of patient 25. LHIS with typical dumbbell shape (black arrows). Patient showed severe pulmonary emphysema.

 

   Discussion
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 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
LHIS, also called "massive fatty deposits"14 or "lipomatous hamartoma,"15 is a well-known but uncommon finding with the accumulation of adipose tissue in the interatrial septum. After the first postmortem description in 1964,16 about 200 cases have been reported. Histologic studies in affected patients typically show hyperplasia of mature multivacuolated fat cells. Fetal fat, hypertrophied myocytes, myocardial fibers, and fibrosis in various quantities may be found.2 14 Although many theories have been suggested, the etiology of LHIS is still unknown. An explanation might be the existence of embryonal mesenchymal cells within the primitive atria forming the interatrial septum that can develop into adipocytes with an appropriate stimulus.13 17

The incidence of LHIS is not known. Although the diagnosis of LHIS is rarely made during a person’s lifetime, due to a lack of typical clinical symptoms, autopsy findings have suggested that the lesion is far more common. The reported incidence of LHIS at autopsy is about 1%,14 18 whereas transthoracic echocardiography studies revealed incidences of up to 8%.19 The results of our study showed an incidence of 2.2% in our patient group, indicating that LHIS has a higher incidence than previously suggested by autopsy. In our opinion, this is in part due to the technical advances of modern CT imaging. In 1982, the first patient was presented in whom the diagnosis of LHIS had been established during life by the use of CT scanning.8 Even conventional CT scanning is able to depict the exact morphology of LHIS, including its characterization as fatty.4 6 13 MSCT scanning represents an enormous technical advance compared to conventional spiral CT scanning. By acquiring multiple sequences simultaneously, shorter data acquisition times, greater coverage, decreased number of motion artifacts, and improved sharpness of images can be achieved. Furthermore, isotropic data sets provide the opportunity to reconstruct images in every axis, thus providing a precise 3-D evaluation of cardiovascular morphology (Fig 2) .

It has been suspected that LHIS is typically associated with obesity and advancing age,1 2 3 18 whereas some authors2 14 have reported that it predominates in women. Our study supports the theory of a coincidence with overweight and advanced age, indicating that the deposition of interatrial fat increases with body weight and age. In our opinion, the slight female predominance in our patient group is not significant.

The diagnosis of LHIS is most commonly made incidentally. In 1969, Kluge20 observed that the presence of LHIS can cause arrhythmia. Since then, some case reports have shown that LHIS might lead to a variety of rhythm disturbances such as P-wave abnormalities, atrial fibrillation, and even sudden death.1 14 21 22 23 Malignant cardiac arrhythmias may occur as a result of extensive bleeding into the lesion.21 Our results revealed a total of 13 of 21 patients (61.9%) with abnormalities found on ECGs. Atrial arrhythmias were the most common finding (8 of 13 patients), which is in agreement with a previous report.1 The mechanism of how LHIS causes cardiac rhythm abnormalities is still unknown, and a clear cause-and-effect relationship has not yet been established.24 Nevertheless, arrhythmia in LHIS may be due to atherosclerotic coronary artery disease, a common finding in affected patients, as shown in our group and in other studies.1 14 In addition, it might be suspected that the involvement of the interatrial septum and the wall of the right atrium, especially in patients with large lesions, interferes with the architecture of atrial myocytes, which could affect conducting pathways.

LHIS presents with characteristic features in imaging studies. It derives exclusively from the upper and/or lower part of the interatrial septum with typical sparing of the foramen ovale, giving the lesion the characteristic dumbbell shape (Fig 1 , 2) . In contrast to other studies that showed the dumbbell shape in every examined patient on transaxial images,13 we found a total of 92.9% of affected patients who shared this feature on transverse images, indicating that this is a typical, but not obligatory, image finding. However, the dumbbell shape was documented in every patient after the acquisition of 3-D reconstructions, including sagittal and coronal views (Fig 2) . None of our patients revealed contrast enhancement of the interatrial mass. The thickness of LHIS in our patient group ranged from 20 to 62 mm, with an average thickness of 35 mm, which is in agreement with former studies.2 14 A common additional finding, as we observed in 21 of 28 patients (75.0%), is an increase in the amount of epicardial and/or mediastinal fat (Fig 3) .13

The first in vivo descriptions of LHIS were made by echocardiography.4 5 6 19 Echocardiography is a noninvasive method used to diagnose intracardiac tumorous lesions, but it is not able to differentiate between fat and connective tissue. Furthermore, the use of transthoracic ultrasound in patients with LHIS is limited due to the accompanying emphysema, which results in restricted acoustic access to mediastinal structures. In our series, 64.3% of patients with LHIS had pulmonary emphysema diagnosed on CT scans. The correlation of these findings, which have been mentioned before,7 is unclear. It might explain why a substantial percentage of patients with LHIS is missed in transthoracic echocardiographic studies.19 It is important to notice that 12 of 18 patients (66.7%) with pulmonary emphysema received systemic steroids, a fact that is known to be a predisposing factor for developing increased mediastinal and, potentially, intracardiac fatty deposits.

The differentiation of LHIS from other cardiac neoplasms based on conventional imaging findings might be difficult. Myxomas are the most common primary cardiac tumors, accounting for 30 to 50% of all cases.24 25 26 The majority of myxomas are solitary and are located in the atria. They arise from the interatrial septum in the vicinity of the foramen ovale, whereas the foramen ovale is always spared in LHIS. Most myxomas are pedunculated on a fibrovascular stalk, thus differentiating it from LHIS, which, as in all of our patients, appears as a homogenous mass with smooth margins. Cardiac lipoma is a true neoplasm occurring in younger patients. In our series, none of the affected patients was < 57 years of age. Lipomas are encapsulated, which is never seen in LHIS. Rhabdomyomas and fibromas are common cardiac tumors in infants and children, and usually occur in the ventricles.24 Cardiac liposarcoma, a rare entity that predominantly appears in the right atrium, is a rapidly growing tumor with early signs of local invasion and hemodynamic comprise. Intraseptal cardiac liposarcomas have never been described. Cardiac metastases are much more common than primary cardiac tumors and may occur in any of the cardiac chambers.26 Their incidence is high in malignant melanoma, lymphoma, and leukemia, in which they occur in the setting of extensive disease. Metastatic malignant disease was not found in any of our patients.

LHIS can be associated with metabolic disorders such as cerebrotendinous xanthomatosis27 or mediastino-abdominal lipomatosis.28 29 Another predisposing factor for developing LHIS is long-term parenteral nutrition, which determines the need for screening those patients.3

LHIS usually does not cause blood flow obstruction, which has been scarcely reported.30 31 In one of our patients who underwent MRI (patient 6), the lesion was seen protruding into the right atrium and superior vena cava, causing hemodynamic impairment. We believe that MRI is a useful method to assess both cardiac anatomy and function in patients with LHIS.32 33

MSCT scanning is a useful method to diagnose LHIS, a benign heart condition that was found to be associated with advanced age, obesity, pulmonary emphysema, and atrial arrhythmias, reaching a 2.2% incidence in our series. Imaging techniques help to differentiate LHIS from other intracardiac masses, thus limiting the need for biopsy and histologic confirmation. In the setting of LHIS, operative intervention should be limited to patients with severe superior vena cava/right atrium obstruction necessitating surgical removal.


   Footnotes
 
Abbreviations: BMI = body mass index; LHIS = lipomatous hypertrophy of the interatrial septum; MSCT =multislice CT; 3-D = three-dimensional

Received for publication November 12, 2003. Accepted for publication March 28, 2003.


   References
TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

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