Δεξιά Καρδιοκαθετηρία και Πνευμονική Υπέρταση

Right Heart Catheterization and Pulmonary Hypertension

• The presentation discusses right heart catheterization and the diagnosis of pulmonary hypertension.
• A 40-year-old patient presented with progressive shortness of breath (SOB) over two years, along with ankle and abdominal swelling. The patient's mother and grandmother died young, suggesting a possible familial component.
• The presentation included various diagnostic imaging studies, including CT angiogram, echocardiogram, showing high pulmonary pressures. Diagnosis is a complex process and not always clear-cut, involving several potential scenarios.

Clinical Classification of Pulmonary Hypertension

• Pulmonary arterial hypertension (PAH) is classified into 5 groups.
• Group 1: Pulmonary arterial hypertension (PAH) - Sub-types include idiopathic, heritable, and associated with drugs/toxins or connective diseases.
• Group 2: PAH associated with left heart disease. Further breakdown includes specific types of heart failure.
• Group 3: PAH associated with lung diseases, and/or hypoxia. Types include obstructive and restrictive lung diseases.
• Group 4: Associated with obstructions in the pulmonary artery. This includes chronic thromboembolic pulmonary hypertension.
• Group 5: Group where the cause of pulmonary hypertension is unclear and/or multifactorial. This can include several underlying conditions.

Pathogenesis of PAH

• Increased pulmonary vascular resistance and raised pulmonary artery pressure.
• Vascular proliferation and remodelling in the pulmonary arteries.
• Distal muscularisation of normally non-muscular arteries and increased muscularisation of muscular pulmonary arteries.
• Formation of plexiform lesions.

Basis of Grades of Hypertensive Pulmonary Vascular Disease

• The presentation highlights the progressive nature of pulmonary hypertension, from cellular changes leading to fibrous and fibroelastic reactions and ultimately plexiform lesions.

The Malignant Nature of PAH

• Angiogenesis & evasion of apoptosis; insensitivity to anti-growth signals are shared features with cancer.

The Rapidly Progressive Nature of PAH

• Reversible stages of the disease including Adventia, Media, Intima, are followed by irreversible stages of the disease, including Thrombosis, Medial hypertrophy, Plexiform lesions.
• The disease progresses from a pre-symptomatic stage through symptomatic and decompensated stages.

Cardiopulmonary Physiology

• The presentation highlights different factors that can contribute to cardiopulmonary issues including conditions that obstruct or restrict airflow or perfusion (like pulmonary arterial hypertension or myoskeletal restriction).
• The relationship between lung ventilation, lung perfusion, heart function, oxygen transport in the blood, and oxygen delivery to the peripheral tissues is noted.
• Different factors that may affect this flow, such as anaemia, skeletal muscle issues (or obesity), valve diseases, endothelial dysfunction and detraining are shown.

Prognosis in PAH

• PAH has an extremely poor prognosis.
• Median survival is 2.8 years; survival rates decrease over time.

Survival in Patients with Untreated PAH

• Survival is affected by different underlying causes of pulmonary arterial hypertension. Survival rates vary depending on the primary cause of PAH.

A Small Delay in Diagnosis

• Delays in diagnosis for various conditions (pulmonary hypertension, lung cancer, advanced colorectal cancer, advanced breast cancer and ischemic cardiomyopathy), have a significant impact on prognosis.

French Registry: Conditions Associated with PAH

• Idiopathic PAH is the most common condition.
• Connective tissue diseases, congenital heart diseases, portal hypertension, appetite suppressant exposure, HIV infections and familial syndromes, are among others associated with PAH.

Incidence and Prevalence of PAH

• The presentation focuses on the incidence and prevalence of idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disorders (CTD), and PAH associated with congenital heart disease (CHD).
• Different conditions, such as HIV, can also increase the risk of PAH.
• Overall prevalence figures are mentioned in the range of 30–50/million/year.

Finding a Needle in a Haystack

• PAH is frequently asymptomatic until it has advanced.
• Symptoms may be vague and overlap with other illnesses, making it challenging to identify. Frequent symptoms are breathlessness, fatigue, and weakness.

PAH Diagnosis—a High Index of Suspicion

• Symptoms of PAH are usually insidious and non-specific.
• Patients exhibiting unexplained dyspnoea, fatigue, or exercise limitation during physical exertion should be investigated for PAH.
• Patients with a family history of PAH , or conditions associated with high PAH prevalence such as connective tissue disorders (CTD) should be considered for evaluation; early diagnosis are crucial for effective management.

Diagnosis of PAH is Typically Delayed

• The presentation highlights that PAH is often diagnosed late. This is attributed to several factors, including low prevalence, low suspicion, symptom manifestation and non-specific symptoms, making diagnosis challenging.

UK and EIRE Specialist Centres

• The presentation lists specialized centers for PAH diagnosis and treatment in the UK and EIRE.

Definition of PH

• The standard definition of pulmonary hypertension includes mean pulmonary artery pressure (mPAP) above a threshold (e.g., 25 mm Hg).
• This threshold value is used to diagnose the disease.

Haemodynamic Characteristics of PH

• Various aspects of PAH are described in terms of haemodynamics and pathophysiology, and different types of pulmonary hypertension using the associated mPAP, PAWP, and PVR (pulmonary vascular resistance) values. This includes pre-capillary and post-capillary pulmonary hypertension.

Pulmonary Hypertension

• Prevalence of pulmonary hypertension is approximately 1% of the global population.
• Different classifications of pulmonary hypertension based on haemodynamics are highlighted.
• Medical, and surgical therapies for different subgroups of pulmonary hypertension are also discussed.

Diagnostic Algorithm of Patients

• The presentation details a diagnostic algorithm for patients with unexplained exertional dyspnoea and/or suspected pulmonary hypertension.
• This includes initial evaluation by a physician, possible referral based on assessment results and further diagnostic testing if required (such as echocardiography).

Modified Bernoulli Equation

• This equation describes the relationship between pressure difference, velocity and other factors important to diagnoses.

Echocardiographic Variables

• The presentation identifies echocardiographic variables that point to the possibility of PH. Findings like high rates of tricuspid regurgitation velocity and right ventricular dysfunction are included in this segment.

Diagnosis of Pulmonary Hypertension

• The steps in diagnosis of pulmonary hypertension, including pulmonary function tests, blood gas analysis, echocardiography, and right heart catheterization, are explained.

CTEPH Diagnostic Algorithm

• The presentation outlines an algorithm for diagnosing chronic thromboembolic pulmonary hypertension (CTEPH), emphasizing the use of right heart catheterization (RHC) and other tests.

Measurement of RHC

• The presentation emphasizes that RHC is the definitive method for diagnosing PAH, by measuring certain pressures and conducting other procedures like testing the vascular resistance, detects shunts and asses associated systemic issues.

Recommendations for Right Heart Catheterization

• Right heart catheterization (RHC) is crucial for confirming and managing pulmonary arterial hypertension.

When? How Often?

• Recommendations for when and how often to perform various assessments like medical assessment, including 6MWT, blood testing (including BNP), ECG and echocardiography or CMRI for evaluating pulmonary hypertension.

RHC in Risk Stratification

• The presentation evaluates the use of right heart catheterization (RHC) to stratify risk in patients with pulmonary hypertension. The criteria for low, intermediate, and high-risk patients are detailed based on clinical findings and objective testing.

Right Heart Cath

• Details about the procedure of right heart catheterization (RHC) are presented.

Pulmonary and Systemic Vascular Resistance

• The presentation describes calculations used to evaluate pulmonary and systemic vascular resistance based on pressures and flow measurements from RHC, for assessing the systemic and pulmonary circulation and related issues.

PAH-CHD: Most Common Type

• The presentation notes the prevalence of pulmonary arterial hypertension (PAH) with congenital heart disease (CHD), highlighting it as a frequent subtype. The information is presented as figures highlighting the relative proportions of various diagnoses.

Case 1 and 2

• Two case presentations are included in the presentation, highlighting possible conditions that could lead to pulmonary hypertension or similar circumstances.

Cardiac Output Measurement Techniques

• Several techniques for measuring cardiac output are reviewed: Fick oxygen method and Indicator dilution methods (indocyanine green and thermodilution), and CMR.

Fick Principle and Oxygen

• The Fick principle for calculating cardiac output is described, including the calculation of oxygen consumption and arteriovenous differences in oxygen across the lungs.

CCM Medgraphics VO2

• The presentation describes methods using Medgraphics machine to measure oxygen uptake. This is a crucial, direct Fick method for assessing cardiac output.

Shunt Detection and Measurement

• Methods using oximetry runs (measurement of blood oxygen saturation) are highlighted to identify and quantify left-to-right shunts. This includes calculations and diagrams.

Mixed Venous Sats

• Method to measure mixed venous saturation (for determining systemic venous oxygen and carbon dioxide levels) is explained using diagrams and calculations.

Oximetry Run

• Diagrams and descriptions of using oximetry measurements to analyze and detect shunts are included.

Pressure Measurement: Wedges

• The presentation explains how wedge pressure measurements are used to assess pulmonary capillary wedge pressure (PCWP) using a catheter and associated equipment. This provides information on related conditions, and how to interpret associated issues and diagnoses.

Right Heart Catheterizations Abnormalities in PCWP Tracing

• Discussion of circumstances when PCWP (pulmonary capillary wedge pressure) readings do not accurately reflect left ventricular end-diastolic pressure is discussed in detail. This may be caused from structural or other conditions.

Recommendations on Fluid Challenge for Unmasking HFpEF

• Guidelines regarding fluid administration tests for patients with possible heart failure are mentioned. Caution is advised concerning use of fluid challenges in diagnosis.

Pulmonary Hypertension due to Left Heart Disease

• Features differentiating high, intermediate, and low likelihood of left heart disease in patients with pulmonary hypertension are described.

Postcapillary Pulmonary Hypertension: Treating the Left Heart

• The approach to treating PAH due to a left-sided heart condition is outlined. This includes improving management of the underlying left heart disorder.

Shunt Detection and Measurement

• The presentation utilizes an oximetry run to visually identify and describe characteristics of shunts for use in assessment.

ASD with Significant [Left-to-Right] Shunt

• Cases of significant left-to-right shunts associated with atrial septal defects (ASDs) are presented, with diagrams and description of the findings.

Shunt Detection & Measurement

• This section describes how to determine blood flow in the patient when left-to-right shunting is present.

Mixed Venous Sats

• Information regarding measurements of mixed venous saturations, to evaluate true venous saturation and help in diagnosis.

Oximetry Run

• The presentation uses diagrams of the heart and lungs, to describe methods of measuring and assessing shunts.

Cardiac Output Measurement Techniques

• Methods such as Fick-oxygen, indicator-dilution (indocyanine green and thermodilution) and CMR methods to measure cardiac output. This section emphasizes the importance of these techniques for making clinical diagnoses.

Thermodilution Method

• The presentation discusses the technique of thermodilution for measuring cardiac output, including its advantages and potential errors.

Fick Principle and Oxygen Measurements

• Fick's principle is described for clinical assessment in details.

CCM Medgraphics VO2

• The use of Medgraphics equipment and their capabilities for analyzing and determining oxygen uptake (VO2) is discussed.

Shunt Detection via Oximetry: Fick Method

• Application of the Fick principle and oximetry measurements method to detect/measure shunts.

Pulmonary and Systemic Vascular Resistance

• The presentation discusses the calculation of pulmonary and systemic vascular resistance using the related formula.

PAH-CHD: Frequency of various types of PAH

• The presentation presents data (possibly from studies or clinical records/registries) illustrating the prevalence of different types of pulmonary arterial hypertension.

Case 1 Summary

• This section provides data and findings (likely from RHC and related tests) from one case study of a patient with possible pulmonary hypertension.

Case 2 Summary

• This section presents the background, diagnostic findings, and interventions performed or implemented in one case study (possibly from records/clinical case presentations).