(II-1A.4d) 47 years old female with Patent Ductus Arteriosus and Pulmonary Hypertension. Jakub Stępniewski MD, Grzegorz Kopeć MD, PhD, Hanna Dziedzic-Oleksy MD, Anna Tyrka MD, Prof. Piotr Podolec MD, PhD

Background
Ductus arteriosus (DA) is a vascular connection between the main pulmonary artery and the aorta that allows blood to bypass the fetus’s non-functioning lungs. After delivery, the DA undergoes active constriction and obliteration eventually. A patent ductus arteriosus (PDA) occurs when the DA fails to close postnatally. PDA is an uncommon clinical finding in adult population. Improvement of survival of premature infants at risk for PDA and an increase in cases discovered incidentally, the incidence of PDA is rising and is estimated at the level of 2.9 per 10,000 live births [1]. There is a female predominance for PDA with a 2:1 female to male ratio [1]. The clinical manifestations of a PDA are numerous and are determined by the degree of left-to-right shunting. It is dependent upon the size and length of the PDA, and the difference between pulmonary and systemic vascular resistances. Hemodynamically PDA can be silent, small, moderate or large. Initially left-to-right shunting causes left ventricle overload. Over time, progressive elevation of pulmonary arterial pressure and rise of pulmonary arterial resistance causes flow reversal (right-to-left shunt) and a cyanotic heart diseases, an Eisenmenger syndrome develops. At this stage prognosis is poor.

Case presentation
We present a case of a 47-year old woman with PDA, who was referred to our Centre for detailed cardiological evaluation and further management. A congenital condition of the heart had been suspected since childhood – abnormal murmurs audible over the heart. No dedicated check-up, however had she undergone until 2012. She has been experiencing progressive decrease of exercise tolerance throughout the years and increase of exertional dyspnea. In April 2012 the diagnosis of PDA was specified by echocardiographic examination, which was scheduled by her occupational doctor, when she was applying for a new job. She was than directed to our Centre. At admission she was hemodynamically stable, with the heart rate (HR) of 75 bpm and the blood pressure (BP) of 140/70 mmHg and no signs of pulmonary congestion or peripheral oedema. There was no cyanosis present. She was considered to be in functional class II by NYHA. An apical impulse was palpable. Continuous murmur grade 4/6 was audible over the base of heart and bounding pulses on the peripheral arteries. Oxygen blood saturation (SatO2) was 95% symmetrical on all extremities. Biochemical analysis showed only mildly elevated NT-proBNP levels up to 869 pg/ml and elevated total cholesterol and LDL values (5,38 mmo/L and 3,5 mmol/L, respectively). Her body weight was 69 kg and her height 162cm (BMI – 26,2). Her exercise capacity was considered moderately impaired – she reached 420 m of distance in 6-minute walking test and had maximal oxygen consumption of 16,3 ml/(kg*min) in a cardiopulmonary exercise test (CPExT). Electrocardiographic (ECG) study showed sinus rhythm 60 bpm, no axis deviation and non-specific intra ventricular conduction defect. Cardiac echo study revealed enlargement of all heart chambers, dilation of the main pulmonary artery (MPA) up to 33 mm with elevated pulmonary valve gradient (18/8 mmHg), presence of a retrograde flow in the left pulmonary artery and the MPA with maximal velocity of 3,7 m/s and the gradient of 48 mmHg, elevated right ventricular systolic pressure (RVSP) up to 112 mmHg, elevated pulmonary flow with Qp/Qs ratio of 3,1:1, restrictive mitral flow pattern with E/A ratio of 2,1 and E/E’ of 20 and a normal left ventricle ejection fraction (LVEF) of 65%. (Table 1) Right heart catheterization (RHC) disclosed pulmonary arterial hypertension (PAH) with mean pulmonary arterial pressure (mPAP) of 86 mmHg and pulmonary arterial resistance (PAR) of 8 Wood units, left-to-right shunt with the Qp/Qs ratio of 2,5:1 and a decrease of mPAP and PAR after nitric oxide (NO) inhalation. (Table 2) No coronary arteries disease was detected on coronary angiography. Cardiac Magnetic Resonance (CMR) showed enlargement of the heart chambers, dilation of the MPA and the presence of PDA of 3 mm of length and 9×9 mm of diameter. (Table 3) Thoracic aorta computed tomography (angio-CT) depicted PDA with 16×11 mm dimensions and 9 mm length.
She was put on bisoprolol 5mg 1×1, perindopril 10mg 1×1, indapamidum 1,5mg 1×1 and atorvastatine 20mg 1×1 before the final decision regarding interventional PDA closure.
We referred to the Experts with two questions: Is she a candidate for PDA closure? If so, would surgical or percutaneous procedure be most advisable for her?

Parameter

Value

LVDd/LVSd

70/41 mm

LV EDV

267 mm

IVSDd

11 mm

PWDd

10 mm

RVDd/(4ChV)

29,5/41 mm

LAd/LAa

51x21x78 mm/ 38 cm2

RAd/RAa

58×41 mm/ 20 cm2

LVEF

65%

asc Ao

35 mm

MPA

34 mm

RVSP

112 mmHg

PV gradient

18/8 mmHg

Ductal jet velocity; gradient

3,7 m/s; 48 mmHg

Qp/Qs

2,5 : 1

Table 1. LVDd- left ventricle diastolic diameter; LVSd- left ventricle systolic diameter; IVSDd- interventricular septum diastolic diameter; PWDs- posterior wall diastolic diameter; RVDd- right ventricle diastolic diameter, 4ChV- four chamber view; LAd- left atrium diameter; LAa- left atrium area; RAd- right atrium diameter; RAa- right atrium area; LVEF- left ventricle ejection fraction; asc Ao- ascending aorta; MPA- main pulmonary artery; RVSP- right ventricle systolic pressure; PV- pulmonary valve.

Pressure [mmHg]

REST

AFTER NO INHALATION

RA

13/11/9

9/8/7

RV

147/4/16

PA (trunk)

129/63/86

95/46/68

PCWP

19/17/16

26/38/23

LV

174/5/24

AORTA

184/73/114

178/73/114

Saturation [%]

VCI

73,3%

62,9%

VCS

73,4%

68,2%

RA

74,2%

RV

74,1%

PA

87,1%

92,1%

Left PA (over PDA)

92,2%

Right PA

92,0%

PCWP

99,9%

98,0%

Aorta

95,9%

98,0%

Cardiac Output [l/min]

4,05

3,08

Cardiac Index [l/min/m2]

2,6

1,8

Qp/Qs

2,5:1

5,3:1

PAR [ARU]

645,1

179,7

TPR [ARU]

792,6

285,7

VSR [mmHg]

1854,7

2073

Table 2. RA- right atrium; RV- right ventricle; PA- pulmonary artery; PCWP- pulmonary capillary wedge pressure; LV- left ventricle; VCI- vena cava inferior; VCS- vena cava superior; PDA- patent ductus arteriosus; PAR- pulmonary arterial resistance; TPR- total pulmonary resistance; VSR- vascular systemic resistance.

Parameter

Value

PDA – lenght:

– diam:

3 mm

9 x 9 mm

LVDd/LVSd

71/47 mm

IVSDd

9 mm

PWDd

11 mm

RVDa

26,3 cm2

LAa

38,7 cm2

RAa

20,3 cm2

LVEF

59%

asc Ao

35 x 37 mm

desc Ao

31 x 26 mm

MPA

42 x 42 mm

RPA

28 x 27 mm

LPA

25 x 25 mm

Table 3. PDA- patent ductus arteriosus; LVDd- left ventricle diastolic diameter; LVSd- left ventricle systolic diameter; IVSDd- interventricular septum diastolic diameter; PWDs- posterior wall diastolic diameter; RVDa- right ventricle diastolic area; LAa- left atrium area; RAa- right atrium area; LVEF- left ventricle ejection fraction; asc Ao- ascending aorta; desc Ao- descending aorta; MPA- main pulmonary artery; RPA- right pulmonary artery; LPA- left pulmonary artery.

Current guidelines
The latest ESC Guidelines for the management of Grown-up Congenital Heart Diseases (GUCH) (version 2010) recommends to consider PDA closure in patients with PAH and PAP > 2/3 of systemic pressure or PVR > 2/3 of SVR but still net left-to-right shunt (Qp/Qs > 1,5 or when testing (preferably with nitric oxide) or treatment demonstrates pulmonary vascular reactivity (Class IIb, level C). [2].

References
1. Reller MD, Strickland MJ, Riehle-Colarusso TR et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153:807.
2. Helmut Baumgartner, Philipp Bonhoeffer, Natasja M.S. De Groot, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010; 31: 2915-2957.

 

Expert’s comments:(Written authorization required from each expert)

1. Robert Hammerschmidt, MD, PhD
This patient is not a perfect candidate for PDA closure. She has clinical signs of left-to-right shunt, left ventricular overload and she has developed pulmonary arterial hypertension with high mean pulmonary arterial pressure and pulmonary arterial resistance, which on the other hand reacts well to NO inhalation. Morphology of this PDA shown in computed tomography and CMR suggests that it is suitable enough for device closure.

2. Henryk Siniawski, MD, PhD
Patients symptoms and clinical findings suggest that this patient might benefit from the PDA closure. Although mean pulmonary arterial hypertension is elevated she has not developed Eisenmenger syndrome yet. Therefore, in order to prevent form shunt reversal PDA should be corrected.

3. Krzysztof Bederski, MD, PhD
Closure of the PDA in this patient is recommended. Although, high pulmonary arterial pressures (mPAP) and resistance (PAR) puts her in higher procedural risk. Fall of the mPAP and PAR after nitric oxide inhalation works in favour for PDA closure. Computed tomography shows bigger sizes of the PDA than CMR. Reevaluation of the sPDA size is recommended in order to decide about the method of closure.

4. Lidia Tomkiewicz- Pająk, MD, PhD
The patient is symptomatic with significant PDA. High pulmonary arterial pressure reacts to NO inhalation. Morphology of PDA and patient age suggests that device closure will be beneficial for the patient. After PDA closure the patients should be systematically follow up, especially to monitor clinical state and pulmonary arterial pressure.

5. Prof. Egle Ereminiene, MD, PhD
This patient is in the second physiologic subgroup of pts with APAH-CHD – with moderately elevated PVR and moderate to large systemic to pulmonary shunting with no cianosis at rest. The management with respect to preferred therapeutic approach is really challenging. The patient has haemodinamically significant left to right shunt with severely dilated LV and both – LV systolic dysfunction (LV ESD – 47 mm) and diastolic dysfunction (restrictive mitral flow pattern with elevated PCWP during RHC) with elevated NT-proBNP levels. (Data about the RV function is lacking). Mean pulmonary arterial pressure is high with elevated PA resistance to 8 Wood units and PVR/SVR – 0,43. On the other hand PVR reacts well to NO inhalation. So, in accordance to the guidelines, there is only IIB (level C) recommendation for the closure of PDA. In the event that PDA is closed, the patient should be counseled that there may be perioperative pulmonary hypertensive crises and complications including the need for inhaled NO or extracorporeal membrane oxygenator bridging as well as the need for long term PAH medical treatment.

Expert’s conclusions:
This patient is qualified for PDA closure. Percutaneous intervention will be performed after reevaluation of PDA sizes in available imaging modalities.

Authors:
Jakub Stępniewski MD1, Grzegorz Kopeć MD, PhD1, Hanna Dziedzic-Oleksy MD1, Anna Tyrka MD1, Prof. Piotr Podolec MD, PhD1

Experts:
Robert Hammerschmidt MD, PhD2, Henryk Siniawski MD, PhD2, Krzysztof Bederski MD3, Lidia Tomkiewicz- Pająk MD, PhD4, Prof. Egle Ereminiene MD, PhD5.

1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Deutsches Herzzentrum Berlin, Germany
3Department of Thoracic Surgery in John Paul II Hospital, Krakow
4Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland
5Department of Cardiology. Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania

 

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