Valutazione del neonato con sospetta ipertensione polmonare
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1 Valutazione del neonato con sospetta ipertensione polmonare Cardiologia Pediatrica Seconda Università degli Studi di Napoli A.O. R.N. dei Colli-Monaldi Napoli
2 Hypoxiemic infant Full or near-term neonate O2 test: PaO2 >100mmHg: lung disease PaO mmHg: lung disease or congenital heart disease (CHD) PaO2 < 50mmHg: persitent pulmonary hypertension of the newborn (PPHN) or (CHD)
3 WHO classification of Pulmonary Hypertension Venice 2003 revised Dana Point Pulmonary arterial hypertension Idiopathic PAH Heritable PAH (BMPR2, ALK1..) Drugs and toxins Associated with CTD, HIV, portal hypertension, congenital heart diseases, chronic hemolytic anemia (SSD) and shistosomiasis Persistent pulmonary hypertension of the newborn (PPHN) 1 Pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PHCM) 2. PH with left heart disease Systolic dysfunction Diastolic dysfunction Valvular 3. PH with lung diseases/hypoxemia COPD Interstitial lung diseases Sleep-disordered breathing Altitude exposure Alveolar hypoventilation Developmental abnormalities 4. CTEPH No more distinction proximal/distal 5. Miscellaneous Sarcoidosis, histiocytosis X, Gaucher,..
4 PPHN First described in 1969 (PFC, persistent fetal circulation) PPHN usually presents at or shortly after birth, when the pulmonary vascular resistance (PVR) fails to decrease at birth. The affected infants fail to establish adequate oxygenation during postnatal life and may develop multi-organ dysfunction. Gersony, Circulation 1969
5 PPHN incidence: 2/1000 live birth Walsh-Sukys MC, Pediatrics 2000
6 PPHN The severity of PPHN can run the full spectrum from mild and transient respiratory distress to severe hypoxemia and cardio-pulmonary instability that require intensive care support. Prompt diagnosis and management, including a timely referral to a tertiary care center, can dramatically improve the chances of survival. Although mortality rates for PPHN were reported as 11 34% during 1980s, current mortality rates are <10% at most tertiary care centers. Hageman JR, Am J Dis Child Davis JM, Pediatr Pulmonol Konduri GG, Pediatrics 2004
7 PPHN: management Confirm diagnosis of PPHN Correct underlying abnormalities (hypothermia, acidosis, hypocalcemia, hypoglycemia, polycythemia); Oxygen by hood Conservative mechanical ventilation Trial of hyperventilation If low PO2, trial of rescue therapies Metabolic HFV Surfactant Vasodilators ECMO Alkalosis ino, PGD 2, PGI 2, Tolazoline, Adenosine, new PDE-i
8 PPHN or CHD?
9 Role of echocardiography It is important to perform echocardiography to exclude cardiac disease and to assess cardiac function. Infants with severe persistent hypoxaemia should be assessed by an experienced paediatric echocardiographer.
10 Echo findings in PPHN RV dilation Anomalous value of pulmonary ejection wave (AcT/ET <0.3) PAPs > 35 mmhg Right-to-left (or bidirectional) ductal or interatrial shunt
11 Estimation of Pulmonary Artery Pressure (PAP) Echocardiographic parameters (direct or indirect) Tricuspid Regurgitation Pulmonary regurgitation Ductal Flow Atrial Shunting Other Shunts Cardiac Function and Output
12 g3 g2 Tricuspid Regurgitation The peak velocity of the tricuspid regurgitation (TR) jet is a direct indicator of the right ventricular pressure (and therefore PAP) RV pressure = RA pressure + (4v 2 )
13 Diapositiva 12 g2 n This is the most accurate way of determining PAP. n The jet of blood leaking through the tricuspid valve is interrogated with Doppler giovannella.russo; 12/10/2013 g3 n It is important to know the systemic blood pressure to determine whether PAP is above systemic BP. giovannella.russo; 12/10/2013
14 Pulmonary artery pressure Mean pulmonary pressure = 79 (0.45 AcT) (Mahan) Pulmonary hypertension Vn = AcT >120 msec (PAPm < 25 mmhg) Normal pattern
15 Anomalous value of pulmonary ejection wave (AcT/ET <0.3)
16 Ductal Flow Pure right-to-left flow indicates that PAP is higher than aortic pressure throughout the cardiac cycle. Bidirectional flow occurs when the aortic and pulmonary pressures are approximately equal. Flow is left-to-right during diastole and right-to-left during systole (as the pulmonary arterial pressure wave reaches the duct before the aortic pressure wave). Bidirectional flow is common in healthy babies in the first 12 hours but changes to pure left-to-right when aortic pressures become higher than PA. gc1
17 Diapositiva 15 gc1 The direction and velocity of ductal blood flow can give useful information on PAP. gabriella carrozza; 13/10/2013
18 2 days newborn, severe PPHN (RV pressure 64mmHg)
19 Atrial Shunting Right-to-left atrial shunting reflects right atrial filling (diastolic) pressure or ventricular filling more than right ventricular systolic pressures. Some degree of right-to-left atrial shunting through the patent foramen ovale is common, although it is rare for this to be purely right-to-left (pure right-toleft flow is Totally Anomalous Pulmonary Venous Drainage [TAPVD]). Bowing of the interatrial septum to the left is commonly seen.
20 Other Shunts When the arterial duct in undetectable at echo or there s no evidence of tricuspid regurgitation, look for other shunt (i.e. small VSD)!!
21 Cardiac Function and Output Quantitative assessment of cardiac function may assist with decisions and assessments of the roles of inotropes, inhaled nitric oxide, and other interventions affecting cardiac output.
22 Dilated right chambers
23 TAPVD
24 TAPVD
25 Dilated right chambers
26 Aortic coarctation Valutazione Color-Doppler
27 Dilated right chambers
28 Critical PS with enlarged RV
29 Take - home message (PPHN) PPHN is uncommon. Clinical evaluation, O2 challenge, echo are mandatory for diagnostic work-up. Diagnosis is polyparametric. Echo is necessary to exclude CHD. Therapy: ino No randomized clinical trials but expert opinion
30
31 Elevated PAP is generally associated with decreased pulmonary blood flow and increased pulmonary vascular resistance. Not uncommonly, there is enlargement of the RV and RA, as well as the main pulmonary artery. There may be flattening or even bowing of the interventricular septum to the left if RV pressures exceed LV pressures. As cardiac output is dependent on venous return to the RA and LA, cardiac output (both RVO and LVO) is frequently reduced with PPHN. Severe PPHN may be associated with LVO below 100ml/kg/min (normal ml/kg/min)
32 tapvd
33 TAPVD The RV is enlarged and the LV is underfilled and "squashed". This is common in severe PPHN The RA and RV are grossly dilated, the LV is small, and another structure - the pulmonary venous chamber, PVC, - is seen (usually not as obviously as in this image)
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