COLD-INDUCED REVERSIBLE MYOCARDIAL ISCHAEMIA IN SYSTEMIC SCLEROSIS

doi:10.1016/S0140-6736(89)92088-6

The Lancet

Volume 334, Issue 8661, 26 August 1989, Pages 475-479

https://doi.org/10.1016/S0140-6736(89)92088-6 Get rights and content

Abstract

The effect of cold provocation on myocardial perfusion was studied in 21 patients with systemic sclerosis and 8 healthy controls. The cold provocation was designed not to cause a pain reaction, and no rise in heart rate/blood pressure product occurred during provocation. Myocardial perfusion was assessed by measurement of thallium uptake by imaged single photon emission computed tomography. No patient had clinical evidence of cardiac involvement, but abnormal electrocardiographic (ECG) findings were found in 5. In 12 patients cold-induced reversible perfusion defects were found; 9 of these also had permanent defects. A further 3 patients had permanent perfusion defects but no reversible defects. The permanent and/or reversible perfusion defects were not related to age among the patients and were not seen in any of the healthy controls, whose age distribution was similar. The reversible and permanent defects were not related to other features of systemic sclerosis, nor to the ECG findings. It is concluded that abnormalities in myocardial perfusion are common in systemic sclerosis and may be present without apparent clinical myocardial involvement. A cold-induced vasospastic process in the myocardial circulation might contribute to the development of the patchy myocardial fibrosis seen in patients with systemic sclerosis.

References (28)

Rj Siegel et al.
Left ventricular function at rest and during Raynaud's phenomenon in patients with scleroderma
Am Heart J
(1984)
Ma Sackner et al.
The heart in scleroderma
Am J Cardiol
(1966)
Gr Botstein et al.
Primary heart disease in systemic sclerosis (scleroderma); advances in clinical and pathologic features, pathogenesis, and new therapeutic approaches
Am Heart J
(1981)
Bh Bulkley
Progressive systemic sclerosis: cardiac involvement
Clin Rheum Dis
(1979)
Ma Sackner et al.
The heart in scleroderma
Am J Cardiol
(1966)
Wp Follansbee et al.
The electrocardiogram in systemic sclerosis (scleroderma). Study of 102 consecutive cases with functional correlations and review of the literature
Am J Med
(1985)
Pj Clements et al.
The relationship of arrhythmias and conduction disturbances to other manifestations of cardiopulmonary disease in progressive systemic sclerosis
Am J Med
(1981)
Bh Bulkley et al.
Angina pectoris, myocardial infarction and sudden cardiac death with normal coronary arteries: a clinicopathologic study of 9 patients with progressive systemic sclerosis
Am Heart J
(1978)
Rg Farmer et al.
Prognostic significance of Raynaud's phenomenon and other clinical characteristics of systemic scleroderma: a study of 271 cases
Circulation
(1980)
Jt Ingram
Acrosclerosis and systemic sclerosis
Arch Dermatol
(1958)

Ec LeRoy et al.

Skin capillary blood flow in scleroderma

J Clin Invest

(1971)

Bh Bulkley et al.

Myocardial lesions of progressive systemic sclerosis: a cause of cardiac dysfunction

Circulation

(1976)

Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee

Preliminary criteria for the classification of systemic sclerosis (scleroderma)

Arthritis Rheum

(1980)

Cited by (98)

Association between systemic sclerosis and left ventricle dysfunction: Findings from observational studies
2023, Heliyon
Cardiac involvement is common in systemic sclerosis (SSc) patients. In this study, we aimed to systematically evaluate the relationship between SSc and left ventricular dysfunction (LVD), especially the left ventricular diastolic dysfunction, by ultrasound and cardiac magnetic resonance data.
We searched The Cochrane Library, PubMed and Embase databases collected studies about comparing LVD parameters in SSc patients and controls from establishment to January 2022. Furthermore, we also performed a two-sample MR using summary statistics from genome-wide association studies (GWAS) important LVD parameters, including left ventricular end-diastolic volume (LVEDV), left ventricular mass (LVM) and left ventricular ejection fraction (LVEF).
Our meta-analysis included 31 eligible studies with 1448 SSc patients. According to the results, SSc patients had lower peak of early diastolic flow velocity/peak of late diastolic flow velocity ratio (E/A ratio), E, trans-mitral early filling peak velocity (E′), and left ventricular end-diastolic diameter (LVEDD) compared to controls. The E/E’ ratio, A, left ventricular isovolumetric relaxation time (IVRT), deceleration Time (DT) and left atrial (LA) diameter were higher in SSc patients in comparison with controls. Moreover, we observed that the SSc patients had lower LVEF than controls. And in MR analysis, we also found that SSc was causally correlated with LVEF (OR = 0.9966, 95% CI 0.9935–0.998, P = 0.0398). However, unfortunately, there was no significant correlation between SSC and LVM (OR = 1.0048, 95% CI 0.9919–1.0179, P = 0.4661) and LVEDV (LVEDV OR = 0.9976, 95%CI 0.9888–1.0066, P = 0.6019).
SSc patients had diastolic/systolic dysfunction. However, MR analysis cannot confirm the genetic relationship between SSc and LVDD because of insufficient data. More research is needed to confirm the causal relationship between the two.
Cardiac involvement in systemic sclerosis: Getting to the heart of the matter
2021, Best Practice and Research: Clinical Rheumatology
Citation Excerpt :
Characteristically, the fibrosis of pSHI is distributed throughout both ventricles, independent of coronary artery distribution, and extensive myocardial fibrosis is found in SSc in patients with patent coronary arteries [3]. Further evidence supporting a “cardiac Raynaud's” hypothesis of pSHI was provided by single photon emission computed tomography (SPECT) studies demonstrating inducible myocardial perfusion defects in response to cold or exercise in the absence of coronary artery disease [10–14]. Histopathological studies have demonstrated nonocclusive concentric intimal hyperplasia of myocardial arterioles [2] and in vivo, reduced coronary flow and resistance reserve in patients with SSc has been noted [15].
Primary systemic sclerosis heart involvement (pSHI) is an important disease manifestation that accounts for a significant proportion of systemic sclerosis (SSc)-associated mortality. A broad clinical spectrum of pSHI exists, which ranges from asymptomatic perfusion abnormalities to diastolic dysfunction or acute myocarditis and congestive heart failure. With improving sensitivity of cardiac investigations, it is increasingly recognized that there is a large burden of subclinical cardiac disease in patients with SSc. Early signs of pSHI can be subtle and determining the etiology of cardiac abnormalities from other causes of cardiomyopathy such as hypertension, ischemic heart disease (IHD), and pulmonary vascular disease remain challenging. Early identification of pSHI potentially provides clinicians with a window of opportunity for intervention to avert progression to heart failure. However, optimal screening and treatment guidelines are lacking, and it is an area of much needed further clinical research.
Systemic sclerosis: Clinical manifestations, anesthetic and orthopedic considerations in a patient
2018, International Journal of Surgery Case Reports
Systemic sclerosis is a rare and progressive multisystem autoimmune disorder that is characterized pathologically by vascular abnormalities, connective tissue sclerosis and atrophy of skin and various internal organs (e.g., alimentary tract, lungs, heart, kidney, CNS), and autoantibodies. With an unknown etiology, Scleroderma is a complex polygenetic disease. A recent Genome Wide Association Study (GWAS) confirmed a strong association with the Major Histocompatibility Complex (MHC) and autoimmunity. We provide a case scenario along with a review of the systems involved and challenges physicians can face in dealing with this rare disease.
Our patient, a known case of systemic sclerosis, was admitted with a history of right femur fracture following a fall. We highlight the medical, anesthetic and surgical challenges faced by our team in the management of this patient. We will explain the stages patient faced in treatment process till her death. We combined the case report with detailed literature review of this rare disease.
Systemic sclerosis is a complex disease process with many different levels of system involvement. Patient needs to be reviewed thoroughly in preoperative period by multidisciplinary team and counseled in detail about the difficulties in procedure, risks and complications.
Patient with scleroderma presents a challenge to the surgical team and anesthetist and a multidisciplinary approach should be followed with all of these patients to avoid catastrophic results.
Systemic Sclerosis
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches
Cardiac involvement in systemic sclerosis (SSc) may be either primary or secondary to the impairment of other organs such as the lungs and kidneys. Primary cardiac involvement, which develops as a direct consequence of the disease, may affect all cardiac tunics, endocardium, myocardium, and pericardium. This may conduct to pericardial effusion, arrhythmias, conduction system defects, myocardial ischemia, myocardial hypertrophy, and heart failure. The prevalence of primary cardiac involvement in SSc varies depending on the sensitivity of the diagnostic tools used, but it is ascertained that overt cardiac manifestations are associated with poor prognosis. Microvascular impairment is a pathognomonic feature of SSc, and together with focal fibrosis may involve all cardiac structures. In contrast, the frequency of atherosclerotic coronary artery disease in SSc seems to be comparable to that of the general population. Because cardiac involvement in SSc can be often clinically obscure, a complete routine evaluation should be adopted in every patient, including standard 12-lead electrocardiogram (ECG) and Doppler echocardiography (especially tissue Doppler imaging), while magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies, although they still represent a second level examination, not available everywhere. Additional investigations can be required in suspicion of other manifestations such as conduction and rhythm disorders (24-h Holter monitoring, invasive electrophysiological studies), or myocarditis (cardiac magnetic resonance). Routine screening for subclinical cardiac involvement with more sensitive tools leads to early diagnosis, in order to treat patients in a “window of opportunity” with drugs that may improve myocardial perfusion and function, and possibly limit the progression of the most life-threatening complications.
Prediction of new onset of resting pulmonary arterial hypertension in systemic sclerosis
2016, Archives of Cardiovascular Diseases
Citation Excerpt :
This hypothesis is supported by previous studies with single photon emission computed tomography assessment of myocardial perfusion, which showed ischaemia in systemic sclerosis without any coronary artery lesions [16]. Myocardial scintigraphy demonstrated evidence of reversible ischaemia together with irreversible lesions, and showed inducibility of coronary vasospasm by cold pressor provocation, suggesting both myocardial ischaemia and fibrosis [17]. Vignaux et al. [18] showed an increase in LV function and in myocardial perfusion after administration of nifedipine in patients with systemic sclerosis, suggesting a link between microvascular dynamic reserve and LV function.
Early detection of pulmonary arterial hypertension (PH) is crucial in systemic scleroderma. However, predictors of new onset of resting PH during follow-up (FUPH) have been poorly explored.
To determine whether nailfold videocapillaroscopy (NVC) grade and exercise echocardiographic variables are predictors of FUPH.
We prospectively enrolled 40 patients with systemic sclerosis (age 54 ± 13 years; 68% women). All patients underwent graded semisupine exercise echocardiography and NVC. Baseline resting PH and FUPH were defined as systolic pulmonary arterial pressure (sPAP) > 35 mmHg, and exercise-induced PH (EIPH) as exercise sPAP > 50 mmHg.
Seventeen patients developed EIPH (43%). During follow-up (FU) (25 ± 15 months), 11 patients without baseline PH developed FUPH (28%), all from the EIPH group. Patients with FUPH were significantly older (60 ± 14 vs 50 ± 12 years; P = 0.04), had higher resting and exercise sPAP (30 ± 4 vs 22 ± 5 and 60 ± 12 vs 40 ± 11 mmHg, respectively; P < 0.0001) and a higher exercise E/e’ ratio (9.4 ± 0.7 vs 5.8 ± 0.4; P = 0.0003) and presented more frequently NVC grade > 2 (90% vs 35%; P = 0.0009). After adjustment for age, resting sPAP, exercise sPAP and NVC grade > 2 were associated with maximal resting sPAP during follow-up and FUPH (P < 0.05). Patients with both EIPH and NVC grade > 2 had a very high incidence of FUPH (82%), and both variables remained strongly associated with FUPH after adjustment for age (hazard ratio 11.6, 95% confidence interval 2.4–55.3; P = 0.002).
Exercise echocardiography and NVC can identify a subgroup of patients with systemic sclerosis who are at risk of developing FUPH.
Les facteurs prédictifs d’apparition d’HTAP pendant le suivi (HTAPsuivi), dans la sclérodermie systémique, demeurent mal connus.
Étudier si le stade de la capillaroscopie digitale (CD) et l’échocardiographie d’effort peuvent prédire l’HTAPSuivi.
Quarante patients avec sclérodermie systémique ont été inclus (âge 54 ± 13 ans ; 68 % femme). Tous bénéficiaient d’une échocardiographie d’effort, et d’une CD. L’HTAP de repos était définie par une pression artérielle pulmonaire systolique (PAPs) > 35 mmHg, l’HTAP d’effort (HTAPeffort) par une PAPs d’effort > 50 mmHg.
Dix-sept patients (43 %) développaient une HTAPeffort. Pendant le suivi (25 ± 15 mois), 11 patients sans d’HTAP à l’inclusion, développaient une HTAPsuivi (28 %), tous avaient une HTAPEffort. Les patients avec une HTAPsuivi étaient plus âgés (60 ± 14 vs 50 ± 12 ans ; p = 0,04) avaient une PAPs de repos et d’effort plus hautes (respectivement, 30 ± 4 vs 22 ± 5 and 60 ± 12 vs 40 ± 11 mmHg ; p < 0,0001), un E/e’ d’effort plus élevé (9,4 ± 0,7 vs 5,8 ± 0,4 ; p = 0,0003) et présentaient plus fréquemment un stade CD > 2 (90 % vs 35 % ; p = 0,0009). Après ajustement à l’âge, les PAPs de repos et d’effort ainsi que le stade CD > 2 étaient associées à la PAPs maximale de repos pendant le suivi et à l’apparition d’HTPSuivi (p < 0,05). Les patients avec une HTAPEffort et un stade CD > 2 avaient une incidence d’HTPsuivi très élevée (82 %). Après ajustement à l’âge, l’association de ces paramètres étaient fortement associée à l’HTPsuivi (HR 11,6, IC 2,4–55,3 ; p = 0,002).
Dans la sclérodermie systémique, l’échocardiographie d’effort et la CD sont utiles pour identifier un groupe de patients à risque de développer une HTAPSuivi.
Early Cardiovascular Disease after the Diagnosis of Systemic Sclerosis
2016, American Journal of Medicine
The purpose of this study is to assess risk and time trends of newly recorded myocardial infarction and stroke in cases with systemic sclerosis.
We conducted a matched incident cohort study (1996-2010) among patients satisfying at least one of the following: 1) diagnosis of systemic sclerosis on at least 2 visits within a 2-year period by a nonrheumatologist physician; or 2) diagnosis of systemic sclerosis on at least one visit by a rheumatologist or from hospitalization; as well as receiving no prior systemic sclerosis diagnosis between 1990 and 1995. Ten controls were matched by birth year, sex, and calendar year of exposure from the general population for each case. Incident myocardial infarction, stroke, and myocardial infarction or stroke was recorded from hospital or death certificates. We estimated incidence rate ratios and hazard ratios (HRs) after adjusting for confounders.
Among 1239 individuals with systemic sclerosis and no history of myocardial infarction (83% female, 56 years old), the incidence rate for myocardial infarction was 13.0/1000 person-years vs 4.1/1000 person-years in the comparison cohort. The incidence rate for stroke was 8.0/1000 person-years vs 3.7/1000 among controls. The adjusted HRs were 3.49 (95% confidence interval [CI], 2.52-4.83) and 2.35 (95% CI, 1.59-3.48) for myocardial infarction and stroke, respectively. For myocardial infarction and stroke, the risk was highest within the first year following diagnosis (HR 8.95; 95% CI, 5.43-14.74 and HR 5.25; 95% CI, 2.90-9.53, respectively).
This large general population-based study indicates an increased risk of myocardial infarction and stroke in patients with systemic sclerosis, especially within the first year of diagnosis.

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COLD-INDUCED REVERSIBLE MYOCARDIAL ISCHAEMIA IN SYSTEMIC SCLEROSIS

Abstract

Am Heart J

Am J Cardiol

Am Heart J

Clin Rheum Dis

Am J Cardiol

Am J Med

Am J Med

Am Heart J

Prognostic significance of Raynaud's phenomenon and other clinical characteristics of systemic scleroderma: a study of 271 cases

Circulation

Acrosclerosis and systemic sclerosis

Arch Dermatol

Skin capillary blood flow in scleroderma

J Clin Invest

Myocardial lesions of progressive systemic sclerosis: a cause of cardiac dysfunction

Circulation

Preliminary criteria for the classification of systemic sclerosis (scleroderma)

Arthritis Rheum