DISCUSSION
Congenital heart defects like patent ductus arteriosus cause
left-to-right shunts. This shunt causes the pulmonary vasculature to
undergo pathological changes such as hypertrophy and fibrosis,
consequently causing PAH. Initially, most patients are asymptomatic
until a significant rise in pulmonary arterial pressure occurs and is
thus present in adulthood, as seen in this patient [7]. Exercise
intolerance, palpitations, and arrhythmias are all different
presentations of PAH. Hemoptysis is a rare presentation and is seen in
only 12% of adult survivors, as seen in this case [8]. These
presentations are more pronounced in the presence of an additional
shunt. As noted in this patient, there is another shunt between the
right pulmonary artery and the aorta. To the best of our knowledge, this
is the first anatomic variant of PDA reported and thus warrants a very
patient-centered approach concerning its management.
The workup for hemoptysis invariably depends on the past medical
history, which in our case was not significant despite the excessive
pulmonary hypertension (68 mmHg on echocardiography) seen in this
patient and is a rather rare clinical picture [9]. Chest X-ray (CXR)
remains the best initial test of choice [10]. It determines the site
of bleeding in 45 to 65% of the cases and the cause in 25 to 35%
[11]. The next step has been a subject of debate as to whether
bronchoscopy is better than CT in determining the cause of hemoptysis;
however, recent studies demonstrate the superiority of CT [9]. In a
case of active hemoptysis, such as ours, bronchoscopy can stimulate
coughing and therefore increase the risk of bleeding. For this reason,
it is often safer to delay the bronchoscopy until a definitive diagnosis
is made [9]. A radiologic definition based on CT states that
pulmonary artery hypertension is diagnosed when the pulmonary artery
diameter is more than 28mm [11]. In our case, this is conclusively
proven by a pulmonary artery diameter of 38mm [14]. The utilization
of computed tomography (CT) in the assessment of patients with pulmonary
hypertension can be advantageous in several ways by providing valuable
insights into the potential underlying causes of the condition, such as
lung parenchymal disease or primary cardiac processes, as well as
chronic thromboembolic disease, and can effectively depict the current
condition of the right ventricle, which is a crucial aspect of patient
care in pulmonary hypertension cases. Echocardiography is used to
consider the probability of PAH, and its parameters help to determine
survival outcomes [13].
Embolotherapy is a widely accepted treatment modality for patients with
severe recurrent hemoptysis [8]. Embolotherapy has been proven to
show immediate control of bleeding in up to 90% of patients; however,
it is associated with a significant risk of re-bleeding in approximately
50% of patients in long-term follow-up [12]. Recent studies have
shown that acute presentations, like in this case if treated surgically,
present with more complications in the long term [11]. Studies have
shown that a surgical approach is also associated with high operative
mortality (> 15%) [8]. On the contrary, conservative
management has shown better patient outcomes in some patients [11].
This includes, a combination of cough suppressants, anti-fibrinolyitcs
like tranexemic acid and antibiotics. Studies have shown a significant
reduction in volume and duration of hemoptysis with the use of
antifibrinolytic agents, thus reducing morbitdity[6]. The other
spectrum of management is to control and adequately monitor PAH, which
is the major cause of mortality in patients with multiple left to right
shunts.
As one of the highest concentrations of PDE5 is found in the lungs, the
PDE-5 inhibitor tadalafil was shown to effectively control PAH when
given once a day [15] [16]. Furthermore, it is regarded as the
most economical choice when compared to alternative treatment methods
[17]. Hence conclusively we state that, due to the rare anatomic
variant, as well as pronounced symptoms present in this patient, it
might prove counter-intuitive to plan a surgical approach and have
preferentially selected the aforementioned conservative approach.