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Hyperkinetic Heart Syndrome

The hyperkinetic heart syndrome also referred to as hyperkinetic heart disease or idiopathic hyperkinetic state, is a cardiac disorder of unknown etiology, that leads to increased cardiac output, overactivity and responds positively to the administration of beta-blockers.


Presentation

The hyperkinetic heart syndrome is an idiopathic cardiac condition that involves heart overactivity and hypercontractility, augmented cardiac output and elevated pulse pressure, without an underlying cardiac pathology [1] [2].

It is primarily diagnosed in patients aged 7-48 years and patients are mostly asymptomatic and in good health [3] [4]. Exercise tolerance is normal, and, due to the absence of symptoms, patients are usually referred for a cardiological evaluation for an auscultated heart murmur. The hyperkinetic heart symptom was first described by R. Gorlin in 1959.

A minority of the patients may report palpitations or fatigue, due to co-existing conditions, such as mitral stenosis or congestive cardiomyopathy [4].

Heart Disease
  • Thus, ischemic heart disease, valvular heart disease, arrhythmias, and conduction defects have been assigned a high priority reflected by their location in the first few sections of this book.[books.google.de]
  • […] medical term "coronary heart disease" is presented.[connection.ebscohost.com]
  • disease or infarction may occur in the absence of organic heart disease.[books.google.de]
  • The hyperkinetic heart syndrome also referred to as hyperkinetic heart disease or idiopathic hyperkinetic state, is a cardiac disorder of unknown etiology, that leads to increased cardiac output, overactivity and responds positively to the administration[symptoma.com]
Systolic Murmur
  • Twelve men suffering from the primary hyperkinetic heart syndrome (PHHS) displayed palpitation, rapid and forceful heart action, increased pulsations of the large arteries, cardiac systolic murmur, and the following circulatory values (averages): systolic[ncbi.nlm.nih.gov]
  • Cardiac examination reveals a normal S1 and S2, but an ejection systolic murmur is heard too.[lecturio.com]
Wide Pulse Pressure
  • A diffuse apical impulse and wide pulse pressure are also detected.[symptoma.com]
  • Signs Wide pulse pressure owing to the leaky flow from the aorta to the pulmonary artery. Prominent carotid pulsations. Hyperkinetic cardiac apex. Accentuated S1 while S2 is narrowly or paradoxically split.[lecturio.com]
Pansystolic Murmur
  • Cardiac auscultation reveals a murmur that can be: Mid-systolic heard along the left sternal border Grade 3 pansystolic murmur over the right precordium Grade 3 basal systolic Grade 2 systolic Grade 4 systolic During the physical examination, the patients[symptoma.com]
  • When the defect is small, there is a high-pressure gradient resulting in a continuous pansystolic murmur palpable as a thrill.[lecturio.com]
Papule
  • Erythema Multiforme *: 0-1 : 0.0 % 2-9 : 0.0 % 10-19 : 0.0 % 20-29 : 0.0 % 30-39 : 0.0 % 40-49 : 100 % 50-59 : 0.0 % 60 : 0.0 % Most common drugs for these people *: Diovan: 1 person, 100.00% Top symptoms for these people *: Rash Papular (redness with papule[ehealthme.com]

Workup

According to the first comprehensive study carried out by Goblin and Brachfeld [5], the hyperkinetic heart syndrome is difficult to diagnose and constitutes a diagnosis made on the basis of clinical suspicion.

Cardiac auscultation reveals a murmur that can be:

  • Mid-systolic heard along the left sternal border
  • Grade 3 pansystolic murmur over the right precordium
  • Grade 3 basal systolic
  • Grade 2 systolic
  • Grade 4 systolic

During the physical examination, the patients are found to be normotensive or slightly hypertensive, even though an extreme rise in systolic blood pressure is not expected. A diffuse apical impulse and wide pulse pressure are also detected.

An electrocardiogram usually reveals left ventricular hypertrophy or a right ventricular overload with no apparent cause; a right bundle branch block and a prolonged QT interval may be found to co-exist as well. The cold pressor test is generally expected to be positive, illustrating reduced peripheral resistance. Plain radiographs frequently show an enlarged ventricular shadow.

Right heart catheterization provides a diagnostic clue that greatly aids in the diagnosis of the hyperkinetic heart syndrome: it can detect an increased cardiac output, a finding shared by all patients with the disorder. It can also help to differentiate the hyperkinetic heart syndrome from a ventricular septal defect.

Additional blood tests involving a complete blood count and biochemical markers usually reveal no abnormalities, or the abnormalities that are detected are unrelated to the hyperkinetic heart syndrome.

Treatment
  • Some studies of the known hypertensive popu lation indicate that one-half or less are receiving adequate treatment, and, of those on therapy, only half have their high blood pressure satisfactorily controlled.[books.google.com]
  • Long term treatment of the hyperkinetic heart syndrome with propranolol . American Journal of the Medical Sciences , 270 (3), 465-474.[moh-it.pure.elsevier.com]
  • Prevention, based largely on altering behaviors that contribute to this chronic disease, is now as important a goal as is treatment of those already afflicted.[books.google.de]
  • Treatment of 12 weeks resulted in a reduction of 14mmHg in systolic and 10mmHg in diastolic pressure with a remarkable reduction of heart rate.[jstage.jst.go.jp]

Prognosis
  • Uncomplicated idiopathic hyperkinetic heart syndrome appears to have an excellent long-term prognosis.[ncbi.nlm.nih.gov]
  • Also they found a “benign short-term and 20-year prognosis in the majority of patients with the idiopathic hyperkinetic heart syndrome” with occasions of regression, although they recommended beta-blockade particularly for patients with hypertension ([onlinejacc.org]
  • In pregnancy, it has an excellent prognosis with prompt multidisciplinary management.[oapublishinglondon.com]
  • PMID 24964980 . a b c "Management and prognosis of stress (takotsubo) cardiomyopathy" . www.uptodate.com . Retrieved 2017-10-21 . Akashi, Yoshihiro J.; Goldstein, David S.; Barbaro, Giuseppe; Ueyama, Takashi (2008-12-16).[en.wikipedia.org]
  • References and Links Lifeinthefastlane.com CCC — Prognosis After Cardiac Arrest CCC — Post-cardiac arrest care CCC — Targeted temperature management (TTM) after cardiac arrest Journal articles Mongardon N, Dumas F, Ricome S, Grimaldi D, Hissem T, Pène[lifeinthefastlane.com]

Etiology
  • The hyperkinetic heart syndrome also referred to as hyperkinetic heart disease or idiopathic hyperkinetic state, is a cardiac disorder of unknown etiology, that leads to increased cardiac output, overactivity and responds positively to the administration[symptoma.com]
  • Table 3 lists clinical features suggesting specific etiologies. 2 Table 3: Clinical Features Suggesting Specific Etiologies Reflex syncope Absence of heart disease Longstanding and recurrent syncope After unexpected pain, smell or sight After prolonged[clevelandclinicmeded.com]
  • Both hereditary and environmental factors play a role in its etiology.[lecturio.com]
  • Although the exact etiology of this dysfunction is unknown, the leading hypothesis suggests that increased circulating catecholamines lead to a diffuse cardiac micro vascular vasospasm leading to cardiac wall motion abnormalities and impaired cardiac[ispub.com]
  • On the etiology and prevalence ofdiseases ofthe heart among soldiers,. London: Churchill. 21. Goetz, C.G.; Turner C.M. and Aminoff M.J. editors (1993). Handbook of Clinical Neurology. B.V.: Elsevier Science Publishers. pp. 429-447. 22.[theposturetheory.com]

Epidemiology
  • Post-cardiac arrest syndrome: epidemiology, pathophysiology, treatment, and prognostication. A consensus statement from the International Liaison Committee on Resuscitation.[lifeinthefastlane.com]
  • License: CC BY 2.5 Definition and Epidemiology of Congenital Heart Disease Congenital heart diseases (CHD) are structural heart defects present since birth.[lecturio.com]
  • The epidemiology of childhood hyperactivity. Institute of Psychiatry, London. 48. Szatmari, P., Offord, D.R., and Boyle, M.H. (1989).[emedmd.com]
  • Epidemiology of reflex syncope. Clin Auton Res 2004; 14(suppl 1):I/9–I/17. Abi-Samra F, Maloney JD, Fouad-Tarazi FM, Castle LW. The usefulness of head-up tilt testing and hemodynamic investigations in the workup of syncope of unknown origin.[clevelandclinicmeded.com]
Sex distribution
Age distribution

Pathophysiology
  • One would surmise, on the basis of the aforementioned, that PH and “hyperkinetic heart syndrome” might have something in common, if they do not represent the same pathophysiological entity.[onlinejacc.org]
  • Post-cardiac arrest syndrome: epidemiology, pathophysiology, treatment, and prognostication. A consensus statement from the International Liaison Committee on Resuscitation.[lifeinthefastlane.com]
  • Further research is required into the reason why it affects women more than men and also into the exact pathophysiology underpinning this cardiomyopathy.[inmo.ie]
  • Morbidity is particularly high in the elderly, and is associated with loss of confidence, fear of falling, depression, fractures, and institutionalization. 2 Back to Top Physiology and Pathophysiology The pathophysiology of syncope involves the interaction[clevelandclinicmeded.com]
  • “Orthostatic Intolerance: Potential Pathophysiology and Therapy”. Chinese Journal of Physiology 47 (3): 102. Retrieved on 3 November 2008. 19. a b c d e Hartshorne, Henry (3 June 1863). “On heart disease in the army”.[theposturetheory.com]

Prevention
  • Prevention, based largely on altering behaviors that contribute to this chronic disease, is now as important a goal as is treatment of those already afflicted.[books.google.de]
  • Far too few people realize what the risks are and what can be done to prevent these risks even in the countries where pro grams in hypertension research are active and the full significance of hyper tension is best understood.[books.google.com]
  • Angina pectoris (prevention of attacks).[healthdirect.gov.au]
  • It is also great in preventing cardiac neurosis . It is popular among bodybuilders since it helps them sleep faster and have ample rest for the next day.[polemeds.org]
  • Dizziness and, in particular, syncope during exercise may result from cardiac disorders that prevent the normal rise (or an actual fall) in cardiac output.[quizlet.com]

References

Article

  1. Gorlin R, Brachfeld N, Turner JD, Messer JV, Salazar E. The idiopathic high cardiac output state. J Clin Invest, 38 (1959), pp. 2144–2153.
  2. R. Gorlin. The hyperkinetic heart syndrome. JAMA, 182 (1962), pp. 823–829
  3. Gillum RF, Teichholz LE, Herman MV, Gorlin R. The idiopathic hyperkinetic heart syndrome: clinical course and long-term prognosis. Am Heart J, 102 (1981), pp. 728–734.
  4. Guazzi M, Polese A, Magrini F, Fiorentini C, Olivari MT. Long-term treatment of hyperkinetic heart syndrome with propranolol. Am J Med Sci. 1975 Nov-Dec;270(3):465-74.
  5. Brachfeld N, Gorlin R. Idiopathic hyperkinetic state: a new clinical syndrome. Br Heart J, 22 (1960), pp. 353–360.

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Last updated: 2018-06-21 20:40