Diagnosis and management of primary heart involvement in systemic sclerosis

Purpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3]. Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e. , interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI. Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI. our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9].The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption availablePurpose of reviewIn systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.Papers of particular interest, published within the annual period of review, have been highlighted as:Systemic sclerosis (SSc) is an immune-mediated multiorgan disease characterized by diffuse vascular damage, aberrant activation of immune system with inflammation, eventually leading to skin and internal organs fibrosis [1-3]. In SSc, cardiac involvement may be primary or secondary [2,3]: the primary heart involvement (SSc-pHI) includes cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications [i.e., interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), renal involvement or other non-SSc-specific cardiac conditions]). It may be sub-clinical and must be confirmed through diagnostic investigations [3]. In SSc, pHI is frequent and once clinically evident is harbinger of poor prognosis, representing the leading cause of disease-related death in 30% of patients [2-4]. Therefore, timely diagnosis is crucial to promptly start the appropriate therapy, thus preventing the potential life-threatening early and late stage complications.Despite the growing interest of the medical community, SSc-pHI is often unrecognized due to the silent nature at early stages and to the lack of a defined diagnostic algorithm [2,3]. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal or oesophageal involvement. As a consequence, it is also difficult to estimate the exact prevalence of SSc-pHI. Depending on the diagnostic technique used, clinical incidence of primary cardiac involvement ranges from 15% to 35% [5,6]. To date, initial autopsy studies, documented widespread inflammatory, fibrotic, and vascular myocardial abnormalities in up to 80% of SSc patients, including those without ante-mortem cardiac symptoms [7,8]. This may be due to age fact that an autoptic study usually investigates patients with more advanced disease. However, cardiac investigations have allowed an increasing recognition of a large burden of subclinical cardiac disease in SSc patients [3].Once diagnosed, there is little evidence on how to best manage SSc-pHI because no specific recommendations are available for SSc-pHI, and a shared treatment approach is still lacking [9]. The aim of this narrative review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for SSc-pHI. no caption available