June 1, 1988 THE AMERICAN JOURNAL OF CARDIOLOGY Volume 61 1355

Kuo et a16 have studied normal subjects, and their results support our contention. Furthermore, it is our observation that the spectral waveforms of patients with severe MS invariably have a linear decay. In patients with sinus tachycardia it may be impossible to distinguish early from late diastolic filling and extrap- olate the decay slope of early filling. This was not a problem in the present study because all patients had slow sinus rhythm.

Our principa1 finding is that atria1 filling fraction is small in most patients with MS and sinus rhythm. Thus, it is unlikely that the symptomatic deterioration that often occurs when atria1 fibrillation develops is explained by loss of effectively timed atria1 contrac-

tion. Tachycardia and catecholamine effects are likely to be more important causes.

1. Carleton RA, Graettinger JS. The hemodynamic role of the atria with and without mitral stenosis. Am J Med 1967;42:532-538, 2. Thompson ME, Shaver JA, Leon DF. Effect of tachycardia on atria1 trans- port in mitral stenosis. Am Heart J 1977;94:297-306. 3. Arani DT, Carleton RA. The deleterious role of tachycardia in mitral stenosis. CircuIation 1967;36:511-516. 4. Heidenreich FP. Thompson ME, Shaver JA, Leonard JJ. Left atriol trons- port in mitral stenosis. C&xIation 1969;40:545-554. -- 5. Seizer A. Effects of atrial fibrillation upon the circulation in patients with mitral stenosis. Am Heart J 1960;59:518-526. 6. Kuo LC. Quinones MA, Rokev R, Sartori M, Abinader EG, Zoghbi WA. Quantification of atria1 contributibn to left ventricular filling by pulsed Dapp- Ier echocardiography and the effect of age in normal and diseased hearts. Am J Cardiof 1967;59:1174-1178.

Cardiac Tumors Associated Hereditary Syndromes

HUMBERTO J. VIDAILLET, Jr., MD

T

with

I he 3 most common primary tumors of the heart in children, rhabdomyomas, fibromas and myxomas, cu- mulatively comprise 66% of all primary cardiac neo- plasms in the pediatric age gr0up.l Although the find- ing of cardiac rhabdomyomas in tuberous sclerosis was originally reported by von Recklinhausen in 1862, the association of cardiac fibromas with the nevoid basal cell carcinoma syndrome and the occurrence of cardiac myxoma in “syndrome myxoma” have only recently been firmly established. To my knowledge, there are no previous publications grouping these he- reditary cardiac tumor syndromes. This report alerts physicians that young patients with cardiac tumors may have a familial syndrome with autosomal domi- nant inheritance. The characteristic physical findings that would allow the clinician to identify these patients are outlined.

Cardiac rhabdomyomas are the most common car- diac tumors found in children.’ Many pathologists be- lieve that rhabdomyoma are hamartomas rather than true neoplasms. Mair reports that approximately 30%

of patients with tuberous sclerosis have cardiac rhab- domyomata and the same proportion of patients with cardiac rhabdomyoma have tuberous sclerosis.2 It ap- pears that the cardiac rhabdomyomas associated with tuberous sclerosis are often embedded in the myocar- dium, as opposed to the intracavitary location more commonly found in the sporadic variant. Cutaneous features of tuberous sclerosis include facial angiofibro- mas, hypomeianotic macules, collagenous plaques, un- gual fibromas, cafe-au-lait spots and cutaneous nod- ules.

From the Department of Cardiology, Marshfield Clinic, Marsh- field, Wisconsin 54449. Manuscript received November 30, 1987; revised manuscript received and accepted February 17, 1988.

Cardiac fibromas are connective tissue neoplasms derived from fibroblast and represent the second most common primary cardiac tumors in the pediatric age group. In a review of 250 patients with cardiac fibro- mas, Go&n found that at least 11 of these individuals had the nevoid basal-cell carcinoma syndrome.3 This association is supported by the rarity of each condi- tion. Fibromas are typically solitary ventricular struc- tures occurring in patients younger than 10 years of age. Cutaneous features of nevoid basal-cell carcino- ma syndrome include nevoid basal-cell carcinomas, milia and palmar pits.

Cardiac myxomas, the most frequently encoun- tered primary neoplasms of the heart in patients of all ages, are the third most common cardiac tumors in children. The term “syndrome myxoma” has been used to describe a subset of patients whose cardiac myxomas are associated with pigmented skin lesions and peripheral and endocrine neoplasms. From our review of 66 cases of cardiac myxoma reported in children, at least 15 (23%) had syndrome myxoma.4 As opposed to control subjects with sporadic myxoma, patients with the syndrome are younger and have a much higher incidence of ventricular, multiple, recur- rent and familial cardiac myxomas. Cutaneous fea- tures of syndrome myxoma include lentiginosis, myx- omas, neurofibromas and blue nevi.

It appears unlikely that the observed association of each primary cardiac tumor with a distinct heredi- tary multisystem syndrome occurs as a totalIy ran- dom event. A hypothesis to explain a common ge- netic mechanism that would predispose these individ- uals to a specific cardiac tumor would be only specula- tive.

1. McAllister HA ]r. Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of Tumor Pathology, foscicle 15. Washington DC: Armed Forces Insti- tute of Pathology. 1976:1-29. 2. Mair DD. Cardiac manifestations. In: Gomez MR, ed. Tuberous Sclerosis. New York: Raven Press, 1979:155-16&t. 8. Gorlin RJ. Nevoid basal-cell carcinoma syndrome. Medicine 1987;66:98- 113. 4. Vidaillet HJ Jr, Seward JB. Fyke FE III, Su WPD, Tajik AJ. Syndrome myxoma: a subset of patients with cardiac myxoma associated with pigmented skin lesions and peripheral and endocrine neoplasms. Br Heart J 1987;57:247- 255.