COR PULMONALE

COR PULMONALE

Introductionas pulmonary arterial hypertension (PAH) is the sine qua non of cor pulmonale, we believe that the best definition of cor pulmonale is :

PAH resulting from diseases affecting the structure and/or the function of the lungs;

PAH results in right ventricular enlargement (hypertrophy and/or dilatation) and may lead with time to right heart failure.

The sine qua non: an essential condition or requirement2

Pulmonary Hypertension (PH)

Haemodynamic & pathophysiological conditionMean pulmonary arterial pressure (PAP) 25 mmHg at rest as assessed by right heart catheterizationCan be found in multiple clinical conditions

CO cardiac output; PAP pulmonary arterial pressure; PH pulmonaryhypertension; PWP pulmonary wedge pressure; TPG transpulmonarypressure gradient (mean PAP mean PWP).3

Cor Pulmonale

defined as right heart strain or overload secondary to acute pulmonary hypertension,

often due to massive pulmonary embolism.Acutecharacterized by hypertrophy & dilatation of the right ventricle (RV) secondary to the pulmonary hypertension

caused by disease of the pulmonary parenchyma &/or pulmonary vascular system between the origins of the main pulmonary artery &the entry of the pulmonary veins into the left atrium.Chronic

Even if many pathobiological mechanisms have been identified inthe cells and tissues of patients with PAH, the exact interactionsbetween them in the initiation and progression of the pathologicalprocesses are not well understood. The consequent increase inPVR leads to right ventricular (RV) overload, hypertrophy, and dilatation,and eventually to RV failure and death. The importance ofthe progression of RV failure on the outcome of IPAH patientsis confirmed by the prognostic impact of right atrial pressure,cardiac index (CI), and PAP,8 the three main parameters of RVpump function.The inadequate adaptation of myocardialTable 8 Updated risk level of drugs and toxins knownto induce PAHPAH pulmonary arterial hypertension.ESC Guidelines 2501contractility seems to be one of the primary events in the progressionof heart failure in a chronically overloaded RV. Changesin the adrenergic pathways of RV myocytes leading to reducedcontractility have been shown in IPAH patients6

Etiology Chronic Cor Pulmonale

COPD* (chronic obstructive bronchitis, emphysema &their association)Asthma (with irreversible airway obstruction)Cystic fibrosisBronchiectasisBronchiolitis obliterans

Neuromuscular diseases: amyotrophic lateral sclerosis, myopathy, bilateral diaphragmatic paralysis, etc KyphoscoliosisThoracoplastySequelae of pulmonary TBSarcoidosisPneumoconiosisDrug related lung diseasesExtrinsic allergic alveolitisConnective tissue diseasesIdiopathic interstitial pulmonary fibrosisInterstitial pulmonary fibrosis of known origin

Central alveolar hypoventilationObesityhypoventilation syndrome (formerly Pickwickian syndrome)Sleep apnoea syndromeObstructive Lung DiseaseRestrictive Lung DiseaseRespiratory Insufficient of central origin

Three major groups of diseases:Those characterised by a limitation to airflow

Those characterised by a restriction of pulmonary volumes from extrinsic or parenchymatous origin

Those where the relatively well preserved mechanical properties of the lungs & chest wall contrast with pronounced gas exchange abnormalities which are partially explained by poor ventilatory drive (respiratory insufficiency of central origin).

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PathophysiologyAlveolar HypoxiaHypoxic pulmonary VasoconstrictionIncreased Pulmonary Vascular ResistancePulmonary HypertentionIncreased Right Ventricular AfterloadRight Ventricular HypertrophyRight Ventricular FailureAcidemia

Cor PulmonaleHypoxemiaCapillary Destruction (Emphysema)Polycythemia

DiagnosisThe clinical exam lacks sensitivity & specificity.Clinical sign of RHF:Distended neck veinsHepatomegalyAscitesPeripheral edemaHyperinflation reduces the yield of cardiac auscultation for the classic signs of PH & CP ie, loud P2, S3 gallop, the systolic murmur of tricuspid regurgitation.

ECG ChangesRight Axis deviationP PulmonalRight Bundle Branch Block

Chest X-Ray

TherapyCurrently there is no specific therapy for PH associated with COPD or interstitial lung diseases

Long-term O2 administration The treatment of choice has been shown partially to reduce the progression of PHBut, PAP rarely returns to normal values & the structural abnormalities of pulmonary vessels remain unalteredoxygen was administered at 1-2 liters/min via nasal, (> 16 hours/day)

PAP: pulmonal arterial pressureTujuan tatalaksanaMengoptimalkan efisiensi pertukaran gasMenurunkan hipertensi pulmonalMeningkatkan kelangsungan hidupPengobatan penyakit dasar dan komplikasinya

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Treatment with conventional vasodilators is not recommended may impair gas exchange due to the inhibition of hypoxic pulmonary vasoconstrictionlack of efficacy after long-term use.