A 42-year-old woman affected by pulmonary atresia came to our attention complaining of dyspnea and fatigue for minimal efforts with important desaturation. After assessing her basal functional capacity with a cardiopulmonary exercise test, the patient was enrolled in an extremely individualized rehabilitation program, which entailed a discreet improvement in the quality of life indices, in the absence of side effects. This paper shows that even patients with extremely severe forms of congenital heart disease, when clinical stable, can undergone a tailored cardiorespiratory rehabilitation program. This must be carried out in a monitored environment and under the supervision of expert personnel

Laura Adelaide Dalla Vecchia

Marinella Sommaruga

Maurizio Bussotti

Patrycja Krasinska

Undetermined

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In memory of SaraAbstractA 42-year-old woman affected by pulmonary atresia came toour attention complaining of dyspnea and fatigue for minimalefforts with important desaturation.After assessing her basal functional capacity with a cardiopul-monary exercise test, the patient was enrolled in an extremelyindividualized rehabilitation program, which entailed a discreteimprovement in the quality of life indices, in the absence of sideeffects.This paper shows that even patients with extremely severeforms of congenital heart disease, when clinical stable, can under-gone a tailored cardiorespiratory rehabilitation program. Thismust be carried out in a monitored environment and under thesupervision of expert personnel.Case ReportA 42-year-old woman presented with the following medicalhistory: at the age of 13 months, during a flu syndrome, she devel-oped exertional cyanosis. A tetralogy of Fallot was suspected.Three months later, she underwent a cardiac catheterization thatshowed pulmonary atresia (PA), an interventricular defect (IVD),a right-sided aortic arch, the presence of some non-confluent pul-monary branches, and a pulmonary flow supported by two collat-erals originating from the thoracic aorta at T5 level (the first col-lateral directed to the left and upwards had a severe stenosis short-ly after the origin and supplied the two upper lobes, the secondone was greatly dilated and supplied the lower left lobe). At theage of 2 years, she underwent a first palliative intervention thatproduced a central shunt interposing a duct between the ascendingaorta and the major collateral connected to the left lung.When the patient was 7 years old, because of a central occlu-sion of the shunting duct, she underwent a modified right Blalock-Taussig palliative intervention (connection of the right subclavianartery to the pulmonary arteries through a synthetic tube). Despitemultiple hospitalizations for lung infections, this girl grew up anddecided to become a doctor. She graduated in 1994 and then spe-cialized in Cardiology at the University of Milan.The last thoracic MRI, at 41 years of age, confirmed a rightventricular outflow atresia with the absence of both the pulmonaryarterial trunk and any significant pulmonary branches on the right,some non-confluent pulmonary branches on the left with twolarge, but stenotic systemic pulmonary collaterals on the left orig-inating from the descending aorta, a large systemic pulmonarycollateral circulation on the right with a sub-diaphragmatic originand a well-developed branch coming from the right subclavianartery, an important dilatation of the aortic bulb and the ascendingaorta with a right-sided aortic arch, a moderate regurgitation of atricuspid aortic valve, a moderately dilated and hypertrophic rightventricle with a preserved systolic function, a wide IVD from sep-tal misalignment with an overriding aorta causing a significantleft-to-right shunt (Figure 1).The combination of PA, IVD and major aortopulmonary collat-eral arteries is the most severe form of tetralogy of Fallot. Duringthe intrauterine development, the absence of both an anterogradeflow from the right ventricle and a flow through the arterial ductCorrespondence: Maurizio Bussotti, Cardiorespiratory RehabilitationDepartment, IRCCS Maugeri Clinical Scientific Institutes, Via Camaldoli64, 20138 Milan, Italy.Tel. +39.02.50725245.E-mail: maurizio.bussotti@icsmaugeri.it Key words: Pulmonary atresia; cardiorespiratory rehabilitation; car-diopulmonary exercise test.Authors’ contributions: MB, MS, PK, contribution to the interpretationof the case, and to the conception and design of this paper; LADV,contribution to the drafting the article, manuscript critical revision. Allthe Authors gave their final approval of the version to be published,and the agreement to be accountable for all aspects of the work.Conflict of interest: The authors declare that they have no competinginterests, and all authors confirm accuracy.Ethics approval and consent to participate: No ethical committeeapproval was required for this case report by the Department, because thisarticle does not contain any studies with human participants or animals. Availability of data and materials: All data underlying the findings arefully available.Received for publication: 11 September 2020.Accepted for publication: 14 December 2020.©Copyright: the Author(s), 2021Licensee PAGEPress, ItalyMonaldi Archives for Chest Disease 2021; 91:1605doi: 10.4081/monaldi.2021.1605This article is distributed under the terms of the Creative CommonsAttribution Noncommercial License (by-nc 4.0) which permits anynoncommercial use, distribution, and reproduction in any medium,provided the original author(s) and source are credited.[page 88]                                             [Monaldi Archives for Chest Disease 2021; 91:1605]                                                               Monaldi Archives for Chest Disease 2021; volume 91:1605Effects of an outpatient service holistic rehabilitation program in a caseof pulmonary atresiaMaurizio Bussotti1, Marinella Sommaruga2, Patrycja Krasinska1, Laura Adelaide Dalla Vecchia11Cardiorespiratory Rehabilitation Department, IRCCS Maugeri Clinical Scientific Institutes, Milan; 2Psychology Unit,IRCCS Maugeri Clinical Scientific Institutes, Milan, Italy Non-ommercial use onlyleads to the development of different types of aortopulmonary col-laterals. These differently supply the lung segments, with a potential-ly infinite variety of pulmonary vascularization states at birth. Mostcollateral arteries usually originate from the descending thoracicaorta; however they can also come from the aortic arch, the subcla-vian artery, the distal thoracic aorta, the internal mammary artery,and the left coronary artery. Identifying the sources of pulmonaryblood flow and creating appropriate pulmonary arborization areparamount to the management of this anomaly [1]. Throughout their life, children with PA develop severecyanosis, symptoms related to heart failure and failure to thrive.The natural history of this heart disease carries high mortality witha 60% survival at 1 year, a survival of less than 50% at 10 years,and less than 20% at 20 years [2]. We first evaluated this patient when she self-referred to ourrehabilitation cardiology center after gaining information fromPubMed [3]. Physical examination was notable for important dig-ital and mucous membranes cyanosis, arrhythmic cardiac soundswith frequent premature beats, a 3/6 systolic murmur at the apex,mild lower limb edema, and hepatomegaly. EKG showed sinusrhythm, pulmonary P waves and right bundle branch block. Shewas taking furosemide, spironolactone and warfarin. Despite many years of experience in the rehabilitative treat-ment of severe forms of pre-capillary pulmonary hypertension, thepatient’s request represented a novelty. Therefore, a complete basalevaluation of her clinical condition and functional capacity wasfirst performed. An echocardiographic examination showed adilated aortic root (mm), ascending tract (50 mm) and arch (34mm), a moderate aortic regurgitation into the right ventricle (RV),a right atrial enlargement ( ml), the presence of a large perimem-branous IVD with a bidirectional shunt; normal left ventricular(LV) volumes and wall thickness, but diffuse hypokinesia deter-mining a moderately reduced LV ejection fraction of 43%, a dilat-ed and moderate hypertrophic RV with moderately reduced sys-tolic function (TAPSE mm), a moderate tricuspid regurgitation, theabsence of the RV outflow tract and pulmonary valve apparatus, adilated and non-collapsible inferior vena cava, normal mitral valveapparatus and normal left atrial volume.A cardiopulmonary exercise test (CPET), as recommended[4,5], was used to evaluate the functional status. The CPET wasconducted on a cycloergometer using a ramp protocol (4Watt/min), maximal for the respiratory quotient (RQ) reached, itwas interrupted because of muscle exhaustion at the load of 28Watt. During exertion, frequent isolated and monomorphic ventric-ular premature beats were recorded. An early anaerobic thresholdwas observed, with an oxygen consumption at the peak exercise(peak VO2) equal to 21% of the predicted value. The slope of therelation between the increase of VO2 and the increase of the workrate (ΔVO2/ΔWR slope) was 5.8 ml/watt/min while the pulse O2 atpeak (peak VO2/heart rate) was 5.9 ml/beat. A severely increasedratio between ventilation and carbon dioxide production(VE/VCO2 = 81) with severe desaturation (descent down to 65%)were also measured indicating a major increase in ventilatory inef-ficiency (Figure 2). Overall, we concluded that there was evidenceof a severe limitation of exercise capacity with multiple etiologicalmechanisms, but a so high relationship VE/VCO2, with a negativeintercept, would confirm the prevalence of a pulmonary vascularlimitation. The distance at the 6-minute walking test (6MWT) was300 m (45% of predicted) and the Maximal Inspiratory Pressure(MIP) was 101 cmH2O.We agreed to enroll the patient in a rehabilitation program (RP),that was individually tailored consisting of muscle strengtheningexercises with dumbbell (0.5 kg), inspiratory muscles training usingthe Threshold IMT®, controlled breathing exercises, submaximalaerobic training with cycloergometer and treadmill and mental gaittraining in the hospital garden at variable speeds and slopes. All                                  [Monaldi Archives forChest Disease 2021; 91:1605]             [page 89]                             Case ReportFigure 1. The last thoracic magnetic resonance imaging per-formed by the patient confirming a right ventricular outflow atre-sia with the absence of both the pulmonary arterial trunk and anysignificant pulmonary branches on the right.Figure 2. A severely increased ratio between ventilation and car-bon dioxide production at the CPET.Non-commercialus only[page 90] [Monaldi Archives for Chest Disease 2021; 91:1605]these activities were performed as described before [6], according toa schedule carried out in agreement with the patient, maintaining alow intensity in terms both of the exercise load and of the number ofsessions per week. Actually the aerobic training was conducted at aconstant workload of 10 Watt for 30 min, established on the basis ofanaerobic threshold determined at the preliminary CPET.Between June and December 2017, the patient completed 30sessions at our outpatient clinic. The RP was interrupted for amonth in August (patient’s holidays) and for 20 days in Octoberdue to an acute bronchitis. During the training no serious adverseevents (syncope, severe arrhythmias, emophyte) were observed. Atthe end of the RP, the 6MWT and the MIP were repeated, with awalked final distance of 283 m (with a maximal nadir of SpO2 of69%), and a measure of 114 cmH2O for MIP at the discharge.At each session, the patient was supported by a clinical psy-chologist. The initial clinical psychometric and psychological eval-uation showed slight anxiety (as quantified by the HospitalAnxiety and Depression Scale (HADS) [7]: anxiety 8/21; depres-sion 7/21), but a high perceived distress emerged, associated withthe history of the disease. Appropriate disease awareness, adequateinternal resources and good family support were also noted. Theinitial psychometric assessment of Quality of Life (EuroQol-5D)[8] showed a self-perception of a moderate health’s state (55/100at the Visual Analog Scale). The psychological intervention had theaim: i) to assess and to manage emotional issues (anxiety, depres-sion, and panic attacks especially); ii) to enhance knowledge,awareness, and acceptance of the illness; and iii) to allow personal,interpersonal, family, social, and occupational adaptation to thedisease [9]. The pre-discharge psychometric evaluation indicated aslight worsening of the anxious symptoms, in the presence, how-ever, of a better awareness and new disease management skills(HADS: anxiety 10/21; depression 5/21). The EuroQol-5D at dis-charge indicated the perception of an improvement in her health’scondition (Visual Analogical Scale 75/100).In the following months, the patient resumed exercising as anoutpatient cardiologist, keeping herself in stable health. Althoughshe no longer participated in a rehabilitation program at our Center,she maintained stable contact with our team, periodically visitingus or meeting via chat with our physiotherapists to periodicallyupdate her rehabilitation program.In January 2019, we learned that the patient had been hospital-ized and died of pneumonia.DiscussionIn this case report, we present a holistic rehabilitation approachto a highly disabling heart disease that led to a significant improve-ment of quality of life without any side effect. PA is an extremely complex congenital heart disease burdenedby a high mortality within the first 20 years of life. These patientsexperience from the first years of life a great limitation of theirability to carry out the common daily activities, in face of impor-tant dyspnea and cyanosis. A constant fatigue, numerous physicallimitations, frequent respiratory complications and hospital admis-sions, despite the complex cardiac surgery they go through as chil-dren, represent a heavy burden for these patients [1]. In addition,the awareness of a poor prognosis inevitably leads to extreme psy-chological fragility [2]. Consensus reports have stated that exercise should be encour-aged and regularly performed in these patients, but this is not com-mon practice [10]. A rehabilitation program, that includes nutri-tional and psychological support, aerobic symptom-limited train-ing, muscle strengthening exercises, inspiratory muscles trainingand controlled breathing exercises, can be beneficial in thesepatients, as already suggested/demonstrated in patients withadvanced forms of cardiorespiratory insufficiency who improvetheir functional capacity and quality of life [10]. It should beemphasized that such RP should be performed under strict moni-toring and by experienced personnel [3,6,11]..ConclusionsRehabilitation programs in patients with congenital heart dis-ease should always be encouraged, even in the most complexforms and in the most severe clinical pictures. In agreement withthe patient, each program should be tailored and achieved througha multidisciplinary approach.From this approach, we can expect an improvement at least inthe quality of life indices, if not necessarily in the exercise per-formance.References 1. Presnell LB, Blankenship A, Cheatham SL, et al. An overviewof pulmonary atresia and major aortopulmonary collateralarteries. World J for Ped and Cong Heart Surg 2015;6:630-9.2. Leonard H, Derrick G, O’Sullivan J, Wren C. Natural andunnatural history of pulmonary atresia. Heart 2000;84:499-503.3. Dalla Vecchia LA, Bussotti M. Exercise training in pulmonaryarterial hypertension. J Thorac Dis 2018;10:508-21.4. Miliaresis C, Beker S, Gewitz M. Cardiopulmonary stress test-ing in children and adults with congenital heart disease.Cardiol Rev 2014;22:275-8.5. Inuzuka R, Diller GP, Borgia F, et al. Comprehensive use ofcardiopulmonary exercise testing identifies adults with con-genital heart disease at increased mortality risk in the mediumterm. Circulation 2012;125:250-9.6. Bussotti M, Gremigni P, Pedretti RFE, et al. Effects of an out-patient service rehabilitation programme in patients affectedby pulmonary arterial hypertension: An observational study.Cardiovasc Hematol Disord Drug Targets 2017;17:3-10.7. Zigmond AS, Snaith RP. The hospital anxiety and depressionscale. Acta Psychiatr Scand 1983;67:361-70.8. Balestroni G, Bertolotti G. [EuroQol-5D (EQ-5D): an instru-ment for measuring quality of life].[Article in Italian]. MonaldiArch Chest Dis 2012;78:155-9.9. Bussotti M, Sommaruga M. Anxiety and depression in patientswith pulmonary hypertension: impact and management chal-lenges. Vasc Health Risk Manag 2018;14:349-60.10. Duppen N, Takken T, Hopman MT, et al. Systematic review ofthe effects of physical exercise training programmes in chil-dren and young adults with congenital heart disease. Int JCardiol 2013;168:1779-87.11. Duppen N, Etnel JR, Spaans L, et al. Does exercise trainingimprove cardiopulmonary fitness and daily physical activity inchildren and young adults with corrected tetralogy of Fallot orFontan circulation? A randomized controlled trial. Am Heart J2015;170:606-14.Case ReportNon-cmmercial use only

Effects of an outpatient service holistic rehabilitation program in a case of pulmonary atresia.

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Effects of an outpatient service holistic rehabilitation program in a case of pulmonary atresia.

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