Case presentation History Abandoned at the age of 1,5 y, since - PDF document

4/18/2018 Center for Congenital Heart Diseases Center for Congenital Heart Diseases July 2006 Case presentation History • Abandoned at the age of 1,5 y, since then in a children's home where he 15 yo boy with PAH/CHD stayed at 3000 m altitude in China Cyanotic boy, “nothing could be done for him in Beijing” • presented with hemoptysis Adopted from China at the age of 4 y, Dutch family • Presentation at Dutch pediatric cardiologist (Leiden) who confirmed a • Theresia Vissia-Kazemier, RN MANP moderate ASD type II with right to left shunt and hypoplasia of the left pulmonary artery Dutch National Network for Pediatric Pulmonary Hypertension Echocardiogram: RVP about 140 mmHg, moderate function of the RV, • Center for Congenital Heart Diseases WHO fc III Department of Pediatric Cardiology Medication: furosemide en spironolacton • Beatrix Children’s Hospital, UMCG RHC: Tcsat 86%, systemic RVP 80 mmHg, PVRi 11 WU.m2 • Start bosentan tid 31¼ mg • University Medical Center Groningen University Medical Center Groningen Center for Congenital Heart Diseases Center for Congenital Heart Diseases September 2007 Referral UMCG 2007 Reffered to our PH center Additional studies: ECG: sinusrhythm, Hf 105/min, right axis 120°, RVH, normal repolarization • Echocardiogram: dilated right atrium, RVH, flattening of septum, right-to-left • shunt AFO /ASD II, severe tricuspid insufficiency with RV pressure of 150 mmHg, normal right pulmonary artery, small left pulmonary artery • 6 MWT: 160 m (hand in hand with his dad) Clinically: more dyspnea at excertion, although since start bosentan • Lab results: Hb 10.4 mmol/l, Nt pro BNP 109 ng/l, normal renal- and • improvement of excercise intollerance liverfunction • Weight 12.9 kg (-2,8 Sd), length 99.6 cm (-2,0 Sd) (China growthcurve reference) • No auto-immuundisease, no clotting disease, normal thyroidfunction Clubbing, TcSat 84%, right sided voussure cardiaq, normal 1th and loud 2th • heartsound, grade III/VI systolic regurgitation murmer, pm 4 IC left, normal Conclusion: breathing sounds, no enlarged liver or spleen Severe PAH with suprasystemic pressures, right-to-left shunt through ASD II, WHO fc III • good systemic RV-function, calibre difference right and left pulmonary artery Plan: if situation deterioates hartcatheterisation and polysomnography to exclude sleepapnea, information about perfusion scan from Leiden will follow University Medical Center Groningen University Medical Center Groningen 1

4/18/2018 Center for Congenital Heart Diseases Center for Congenital Heart Diseases Follow up Follow up 2008: 2012-2017: Improvement After 1 year treatment improvement of physical condition, WHO Fc II Stable condition • • Echo: RV ↓ 125 mmHg, MWT ↑ 375 m., NT pro BNP 512 ng/L 2011: Playing with little brother and sister Worsening of clinical situation: Swimming, snorkeling, school, friends Complaints: more dyspnea on excertion, diminshed excercise capacity, • dizziness, WHO Fc III • Nt-pro-BNP 389 ng/L Re-evaluation with RHC: PAP 77 mmHg, PVRI ↓ , 10 WUm2 (stable), cardiac • index ↑ , no R-L shunt • Add on sildenafil uptitrated 3 dd 10 mg University Medical Center Groningen University Medical Center Groningen Center for Congenital Heart Diseases Center for Congenital Heart Diseases February 2018 Follow up March 2017 Admission PICU Leiden Acute event at school: coughing up blood Increase dyspnea, less excercise intollerance Ambulance > tranexamic acid > 500 ml coughing blood •  Ti gradient 177 mmHg, severe RV dilatation, diminshed RV function Transport to Hospital Leiden, admission ICU •  At arrival in Hospital (SEH, intensivist, pediatric cardiology, pediatrician): Transport to PICU Groningen Start optiflow, milirone, add on epoprostenol i.v. PICC line Awake, alert patient, coughing up a lot amount of blood, Rf 40/min, Spo2 75% • with NRM 2 L, RR MAP 70 mmHg, HR 130/min, pain right sided thoracic, Optiflow #, NRM 10-15 l/min, uptitration flolan to 20 ng/kg/min • temperature 36.9 Re-evaluation RHC (combined with insertion port-a cath): • PAP 102 mmHg ↑ , 25 WU*m2 ↑ , cardiac index ↓ Lab: lactate 2.0, Hb 7.7, INR 5.4 > substantial deterioration of PAH Chest X ray: right sided infiltration, bleeding CT scan: no indications for acute or chronic pulmonary embolism • Medication: Morfin 1 mg, Co-fact (Human protrombine complex = factor Start anticoagulation: initial LMWH s.c, followed by acenocoumarol • II/VII/IX/X), vit. K 2 mg INR targed range 2,5-3,5 > On the way to CT scan University Medical Center Groningen University Medical Center Groningen 2

4/18/2018 Center for Congenital Heart Diseases Center for Congenital Heart Diseases Definition and origin of hemoptysis CT scan : active bleeding right upper lob, indication for coiling Definition Hemoptysis is the coughing up of blood or bloody sputum from the lungs or airway. It may be either self-limiting or recurrent In CT room : circulatory instability, hypovolemic shock, MAP ↓ 30 mmHg, start Massive hemoptysis is defined as 200-600 mL of blood coughed up within a noradrenalin 0.05mcg/kg/min, erythrocyte transfusion period of 24 hours or less HC and PVE: right pulmonary artery, identification of bleeding in upper lobe Origin in PAH Diagnostics: branch, embolisation Pulmonary circulation is reduced at the level of the pulmonary arteriole due to Decreasing saturations, probably du to hematohoracid, pleural drain (drainage of Lab (blood count, coagulation, infection) vascular remodeling, hypoxemie vasocontriction and microthrombosis 1000ml blood) Chest X ray Identification of 2nd bleeding branch, attempts to reach this branch failed due to Thoracic HR CT Lack of blood flow gives rise to bronchial artery angiogenesis with subsequent continuously low saturations > intubation and ventilation Angiography enlargement Bronchoscopy (to identify the bleeding) Persistent low saturations > bradycardia and asystolie, resucitation unsuccessful Patient died in presence of his mother These vessel may rupture due to elevated regional blood pressure, and leak into the tracheobronchial tree causing hemoptysis University Medical Center Groningen University Medical Center Groningen Center for Congenital Heart Diseases Center for Congenital Heart Diseases Strategies to treat hemoptysis Dutch national cohort 1993-2012 1 Palareti et al 1996 : Supportive care (antibiotics, oxygen) Total number of patients = 74, 13 (17,6%) children with hemoptysis • About 20% bleeding • Surgical resection (increased mortality) Equally divided iPAH/FPAH (n=7) and PAH/CHD (n=6) events during Bronchial artery embolization > currently most used strategy Life threatening hemoptysis (N=5) more in group iPAH/FPAH (p=0.246) • anticoagulation • Lungtransplant Mean age first hemoptysis 12.5 y therapy occur at very low INR Technique: Longer time since diagnosis (p= 0.001) Injection particulate matter into angiographically to identify bronchial arterial More frequently use anticoagulant therapy (p=0,006) (INR ranged 1.5-2.7) that is bleeding and to attempt hinder further bleeding Cox regression analysis did not identify use of VKA anticoagulant therapy as a - Polyvinyl alcohol (PVA) non absorbable particles 300-500 µm risk factor for the first hemoptysis event Roofthooft et al 2013: - Fibred platinum coils (occlude more proximal vessels) Discontinuation of Recurrent bleeding is reported after embolization Increased risk of hemoptysis with univariate analysis: VKA did not prevent recurrence of (all at the time of diagnosis) Sometimes part of blood supply of anterior spinal artery come from hemoptysis - Older age - Higher mean PAP bronchial vessels > risk of paresis/paralysis causing motor deficits and - WHO fc IV - Higher indexed pulmonary vascular resistance sensory deficits (pain / temperature sense loss) 1 Roofthooft et al. Am J Cardiol 2013;112-1505-1509 University Medical Center Groningen University Medical Center Groningen 3

4/18/2018 Center for Congenital Heart Diseases Center for Congenital Heart Diseases THM Zylkowska et al. 2001/Jaix et al. 2009 In adults with PAH Survival rate of ≤ 50% 3 months after 10/13 pts with hemoptysis died or underwent (H)LTX, in 6 patient directly • hemoptysis related to hemoptysis Hemoptysis is a serious condition, It is unknown if early PVE in non massive hempotysis may prevent or • reduce the risk of subsequent life threatening hemoptysis in case of life threatening hemoptysis with poor outcome Total cohort 1-,3- and 5 year transplant free survival: 84%-71%-62% • Listing patients for transplantation should be considered Non-massive hemoptysis: 75%-57%-48% • even in patients in further hemodynamically stable patients Therefore PVE cannot be regarded as definitive treatment for life • threatening hemoptysis After acute percutaneous vascular embolization (PVE) consideration of • urgent lungtransplantation seems justified University Medical Center Groningen University Medical Center Groningen 4